Podcast
Questions and Answers
In chronic myeloid leukemia (CML), what is the result of the reciprocal translocation between chromosomes 9 and 22?
In chronic myeloid leukemia (CML), what is the result of the reciprocal translocation between chromosomes 9 and 22?
- Inhibition of tyrosine kinase activity
- Deletion of the ABL1 gene
- Formation of the BCR-ABL1 fusion protein (correct)
- Increased production of normal white blood cells
What is the primary mechanism by which the BCR-ABL1 tyrosine kinase contributes to the pathogenesis of chronic myeloid leukemia (CML)?
What is the primary mechanism by which the BCR-ABL1 tyrosine kinase contributes to the pathogenesis of chronic myeloid leukemia (CML)?
- It promotes cell adhesion to the bone marrow stroma
- It inhibits the production of myeloid cells
- It enhances the cell's ability to repair DNA damage
- It continuously activates signal transduction pathways, leading to increased proliferation (correct)
A patient is diagnosed with chronic myeloid leukemia (CML). Cytogenetic testing reveals the presence of the Philadelphia chromosome. What specific genetic event is indicated by this finding?
A patient is diagnosed with chronic myeloid leukemia (CML). Cytogenetic testing reveals the presence of the Philadelphia chromosome. What specific genetic event is indicated by this finding?
- Reciprocal translocation between the long arms of chromosomes 9 and 22 (correct)
- Duplication of the BCR gene on chromosome 9
- Deletion of a tumor suppressor gene on chromosome 17
- Inversion of genetic material on chromosome 21
In the context of myeloproliferative neoplasms (MPNs), what distinguishes atypical CML (aCML) from typical CML?
In the context of myeloproliferative neoplasms (MPNs), what distinguishes atypical CML (aCML) from typical CML?
What is typically observed in the peripheral blood smear (PBS) of a patient with chronic myeloid leukemia (CML)?
What is typically observed in the peripheral blood smear (PBS) of a patient with chronic myeloid leukemia (CML)?
How did the World Health Organization (WHO) classification system change the diagnostic criteria for myeloproliferative neoplasms (MPNs) compared to the previous FAB classification?
How did the World Health Organization (WHO) classification system change the diagnostic criteria for myeloproliferative neoplasms (MPNs) compared to the previous FAB classification?
What is the underlying cause of clonal hematopoietic disorders in myeloproliferative neoplasms (MPNs)?
What is the underlying cause of clonal hematopoietic disorders in myeloproliferative neoplasms (MPNs)?
What is a key characteristic of the BCR-ABL1 fusion gene's function in the pathogenesis of CML?
What is a key characteristic of the BCR-ABL1 fusion gene's function in the pathogenesis of CML?
A patient with CML in the chronic phase progresses to the accelerated phase. Which of the following is most likely to be observed?
A patient with CML in the chronic phase progresses to the accelerated phase. Which of the following is most likely to be observed?
In polycythemia vera (PV), what is the primary abnormality driving the overproduction of red blood cells?
In polycythemia vera (PV), what is the primary abnormality driving the overproduction of red blood cells?
A patient is suspected of having polycythemia vera (PV). Which of the following diagnostic criteria is considered a major criterion according to WHO?
A patient is suspected of having polycythemia vera (PV). Which of the following diagnostic criteria is considered a major criterion according to WHO?
What is the most common genetic mutation associated with polycythemia vera (PV)?
What is the most common genetic mutation associated with polycythemia vera (PV)?
A patient with polycythemia vera (PV) presents with pruritus, especially after a warm shower. What is the underlying mechanism most likely contributing to this symptom?
A patient with polycythemia vera (PV) presents with pruritus, especially after a warm shower. What is the underlying mechanism most likely contributing to this symptom?
What is the goal of therapeutic phlebotomy in the early stages of polycythemia vera (PV) treatment?
What is the goal of therapeutic phlebotomy in the early stages of polycythemia vera (PV) treatment?
A patient with essential thrombocythemia (ET) presents with microvascular thromboses. Which of the following contributes to this complication?
A patient with essential thrombocythemia (ET) presents with microvascular thromboses. Which of the following contributes to this complication?
How is essential thrombocythemia (ET) distinguished from secondary or reactive thrombocytosis?
How is essential thrombocythemia (ET) distinguished from secondary or reactive thrombocytosis?
In the pathogenesis of essential thrombocythemia (ET), which of the following mutations is commonly implicated?
In the pathogenesis of essential thrombocythemia (ET), which of the following mutations is commonly implicated?
What is a typical finding on bone marrow biopsy in a patient with primary myelofibrosis (PMF)?
What is a typical finding on bone marrow biopsy in a patient with primary myelofibrosis (PMF)?
A patient with suspected primary myelofibrosis (PMF) presents with anemia, splenomegaly, and 'teardrop' shaped red blood cells on peripheral blood smear. This combination BEST describes which of the following?
A patient with suspected primary myelofibrosis (PMF) presents with anemia, splenomegaly, and 'teardrop' shaped red blood cells on peripheral blood smear. This combination BEST describes which of the following?
Which of the following is a constitutional symptom commonly associated with primary myelofibrosis (PMF)?
Which of the following is a constitutional symptom commonly associated with primary myelofibrosis (PMF)?
What is the underlying cause of ineffective hematopoiesis in primary myelofibrosis (PMF)?
What is the underlying cause of ineffective hematopoiesis in primary myelofibrosis (PMF)?
Considering the link between polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF), what does the hypothesis regarding the 'dosage effect' of JAK2 TK activity suggest?
Considering the link between polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF), what does the hypothesis regarding the 'dosage effect' of JAK2 TK activity suggest?
Which treatment strategy is typically considered first-line for managing splenomegaly in primary myelofibrosis (PMF)?
Which treatment strategy is typically considered first-line for managing splenomegaly in primary myelofibrosis (PMF)?
A patient with primary myelofibrosis (PMF) has progressive anemia despite supportive care. What therapeutic approach might be considered to alleviate the anemia?
A patient with primary myelofibrosis (PMF) has progressive anemia despite supportive care. What therapeutic approach might be considered to alleviate the anemia?
Which of the following best describes the 'dys' prefix in the context of myelodysplastic neoplasms?
Which of the following best describes the 'dys' prefix in the context of myelodysplastic neoplasms?
What is the primary characteristic that defines myelodysplastic syndromes (MDS)?
What is the primary characteristic that defines myelodysplastic syndromes (MDS)?
A patient is diagnosed with a pre-MDS condition. Which of the following findings would be most consistent with clonal cytopenia of unknown significance (CCUS)?
A patient is diagnosed with a pre-MDS condition. Which of the following findings would be most consistent with clonal cytopenia of unknown significance (CCUS)?
What is a crucial distinction between myelodysplastic syndromes (MDS) and conditions like essential thrombocythemia (ET) or polycythemia vera (PV)?
What is a crucial distinction between myelodysplastic syndromes (MDS) and conditions like essential thrombocythemia (ET) or polycythemia vera (PV)?
A patient previously treated with chemotherapy develops therapy-related myelodysplastic syndrome (t-MDS). Which factor is most significantly implicated in the pathogenesis of t-MDS?
A patient previously treated with chemotherapy develops therapy-related myelodysplastic syndrome (t-MDS). Which factor is most significantly implicated in the pathogenesis of t-MDS?
Why might a patient with myelodysplastic syndrome (MDS) experience increased rates of infection and bleeding?
Why might a patient with myelodysplastic syndrome (MDS) experience increased rates of infection and bleeding?
A bone marrow biopsy from a patient with suspected myelodysplastic syndrome (MDS) shows dysplasia in erythroid and myeloid lineages, but no increase in blasts. Which additional test is most important to confirm the diagnosis?
A bone marrow biopsy from a patient with suspected myelodysplastic syndrome (MDS) shows dysplasia in erythroid and myeloid lineages, but no increase in blasts. Which additional test is most important to confirm the diagnosis?
How does the presence of ring sideroblasts relate to the pathophysiology of myelodysplastic syndromes (MDS)?
How does the presence of ring sideroblasts relate to the pathophysiology of myelodysplastic syndromes (MDS)?
Which of the following abnormalities involving granulocytes would be most indicative of myelodysplastic syndrome (MDS)?
Which of the following abnormalities involving granulocytes would be most indicative of myelodysplastic syndrome (MDS)?
A patient with myelodysplastic syndrome (MDS) is found to have abnormal megakaryocytes in the bone marrow. What hematological consequence is most likely associated with this finding?
A patient with myelodysplastic syndrome (MDS) is found to have abnormal megakaryocytes in the bone marrow. What hematological consequence is most likely associated with this finding?
In an otherwise healthy 70-year-old patient presenting with macrocytic anemia, which of the following peripheral blood smear findings would most strongly suggest a diagnosis of myelodysplastic syndrome (MDS)?
In an otherwise healthy 70-year-old patient presenting with macrocytic anemia, which of the following peripheral blood smear findings would most strongly suggest a diagnosis of myelodysplastic syndrome (MDS)?
A patient's bone marrow aspirate reveals 8% blasts, dysplastic changes in more than one cell lineage, and a normal karyotype. According to the WHO classification, how should the patient be classified?
A patient's bone marrow aspirate reveals 8% blasts, dysplastic changes in more than one cell lineage, and a normal karyotype. According to the WHO classification, how should the patient be classified?
Which of the following best explains the simultaneous presence of proliferation and apoptosis of hematopoietic cells in MDS?
Which of the following best explains the simultaneous presence of proliferation and apoptosis of hematopoietic cells in MDS?
A patient is diagnosed with MDS with isolated del(5q). What is a typical characteristic of this specific MDS subtype?
A patient is diagnosed with MDS with isolated del(5q). What is a typical characteristic of this specific MDS subtype?
In a patient diagnosed with MDS-SF3B1, what cytogenetic or molecular finding is characteristically present?
In a patient diagnosed with MDS-SF3B1, what cytogenetic or molecular finding is characteristically present?
What distinguishes MDS with multilineage dysplasia from MDS with single lineage dysplasia?
What distinguishes MDS with multilineage dysplasia from MDS with single lineage dysplasia?
What is the significance of classifying Myelodysplastic Syndromes (MDS) according to the International Prognostic Scoring System (IPSS) or its revised version (IPSS-R)?
What is the significance of classifying Myelodysplastic Syndromes (MDS) according to the International Prognostic Scoring System (IPSS) or its revised version (IPSS-R)?
In MDS/MPN neoplasms, what genetic abnormality is frequently associated with MDS/MPN with SF3B1 mutation and thrombocytosis?
In MDS/MPN neoplasms, what genetic abnormality is frequently associated with MDS/MPN with SF3B1 mutation and thrombocytosis?
According to the WHO 2022 classification, what is a key difference between myelodysplastic and myeloproliferative chronic myelomonocytic leukemia (CMML)?
According to the WHO 2022 classification, what is a key difference between myelodysplastic and myeloproliferative chronic myelomonocytic leukemia (CMML)?
A patient is suspected of having a myeloid neoplasm. What is the MOST critical factor in differentiating between a diagnosis of MDS and MPN?
A patient is suspected of having a myeloid neoplasm. What is the MOST critical factor in differentiating between a diagnosis of MDS and MPN?
Why are patients with Myelodysplastic Syndrome (MDS) at an increased risk of developing Acute Myeloid Leukemia (AML)?
Why are patients with Myelodysplastic Syndrome (MDS) at an increased risk of developing Acute Myeloid Leukemia (AML)?
Which of the following best explains the role of pyroptosis in the pathophysiology of MDS?
Which of the following best explains the role of pyroptosis in the pathophysiology of MDS?
In the context of MDS, what does the term 'ineffective hematopoiesis' primarily refer to?
In the context of MDS, what does the term 'ineffective hematopoiesis' primarily refer to?
A peripheral blood smear from a patient with suspected MDS shows a population of large, abnormally shaped red blood cells. What is the MOST appropriate term to describe these cells?
A peripheral blood smear from a patient with suspected MDS shows a population of large, abnormally shaped red blood cells. What is the MOST appropriate term to describe these cells?
A 60-year-old patient is diagnosed with refractory anemia. What is the key laboratory finding that would differentiate this from other causes of anemia and suggest a diagnosis of MDS?
A 60-year-old patient is diagnosed with refractory anemia. What is the key laboratory finding that would differentiate this from other causes of anemia and suggest a diagnosis of MDS?
Which of the following is a recognized site of involvement for mature lymphoid neoplasms?
Which of the following is a recognized site of involvement for mature lymphoid neoplasms?
What is a key consideration in the classification of mature lymphoid neoplasms?
What is a key consideration in the classification of mature lymphoid neoplasms?
Which of the following is a recognized 'B symptom' associated with lymphoid neoplasms?
Which of the following is a recognized 'B symptom' associated with lymphoid neoplasms?
In the diagnostic evaluation of lymphoid neoplasms, what is the role of flow cytometry?
In the diagnostic evaluation of lymphoid neoplasms, what is the role of flow cytometry?
What does immunophenotyping contribute to the diagnosis of lymphoid neoplasms?
What does immunophenotyping contribute to the diagnosis of lymphoid neoplasms?
Why is molecular testing considered the gold standard in the diagnosis of leukemias and lymphomas?
Why is molecular testing considered the gold standard in the diagnosis of leukemias and lymphomas?
What is the primary role of biochemical analysis (e.g., serum creatinine, uric acid) in the workup of lymphoid neoplasms?
What is the primary role of biochemical analysis (e.g., serum creatinine, uric acid) in the workup of lymphoid neoplasms?
Within the context of lymphoma diagnostics, what information does the Ann Arbor staging system provide?
Within the context of lymphoma diagnostics, what information does the Ann Arbor staging system provide?
In the context of Non-Hodgkin's Lymphoma, what is the primary utility of the Lugano classification?
In the context of Non-Hodgkin's Lymphoma, what is the primary utility of the Lugano classification?
What implication does the 'mismatch' in antigenic patterns (antigens not found in normal mature cells) have in some lymphoid neoplasms?
What implication does the 'mismatch' in antigenic patterns (antigens not found in normal mature cells) have in some lymphoid neoplasms?
What is a key difference between acute lymphoblastic leukemia (ALL) in children versus adults?
What is a key difference between acute lymphoblastic leukemia (ALL) in children versus adults?
Which of the following is a shared symptom between B-ALL and T-ALL?
Which of the following is a shared symptom between B-ALL and T-ALL?
What genetic abnormality is frequently observed in T-ALL/T-LBL?
What genetic abnormality is frequently observed in T-ALL/T-LBL?
In the classification of Acute Lymphoblastic Leukemia (ALL), what does 'T-ALL, NOS' refer to?
In the classification of Acute Lymphoblastic Leukemia (ALL), what does 'T-ALL, NOS' refer to?
What cytogenetic abnormality in B-ALL/LBL is associated with the worst prognosis?
What cytogenetic abnormality in B-ALL/LBL is associated with the worst prognosis?
What is a key immunophenotypic marker shared between both B-ALL and T-ALL?
What is a key immunophenotypic marker shared between both B-ALL and T-ALL?
Which genetic alteration is associated with a favorable prognosis in B-ALL?
Which genetic alteration is associated with a favorable prognosis in B-ALL?
What is the typical immunophenotype of CLL/SLL?
What is the typical immunophenotype of CLL/SLL?
In CLL/SLL diagnosis, what is the significance of 'smudge cells'?
In CLL/SLL diagnosis, what is the significance of 'smudge cells'?
How does the presence or absence of IGHV mutation relate to the prognosis of CLL?
How does the presence or absence of IGHV mutation relate to the prognosis of CLL?
What is a key feature differentiating Monoclonal B-Cell Lymphocytosis (MBL) from CLL?
What is a key feature differentiating Monoclonal B-Cell Lymphocytosis (MBL) from CLL?
Which of the following is a characteristic feature of T-Prolymphocytic Leukemia (T-PLL)?
Which of the following is a characteristic feature of T-Prolymphocytic Leukemia (T-PLL)?
What is a typical clinical manifestation of Prolymphocytic Leukemia (PLL)?
What is a typical clinical manifestation of Prolymphocytic Leukemia (PLL)?
What is a common feature observed in Hairy Cell Leukemia?
What is a common feature observed in Hairy Cell Leukemia?
What is a key diagnostic feature of T-large Granular Lymphocytic Leukemia?
What is a key diagnostic feature of T-large Granular Lymphocytic Leukemia?
What is a primary mode of transmission for HTLV-1, which is associated with Adult T-Cell Leukemia/Lymphoma (ATLL)?
What is a primary mode of transmission for HTLV-1, which is associated with Adult T-Cell Leukemia/Lymphoma (ATLL)?
A patient is diagnosed with acute Adult T-Cell Leukemia/Lymphoma (ATLL). What is the typical prognosis for this subtype?
A patient is diagnosed with acute Adult T-Cell Leukemia/Lymphoma (ATLL). What is the typical prognosis for this subtype?
In the classification of Adult T-Cell Leukemia/Lymphoma (ATLL), which of the following is a typical immunophenotypic marker?
In the classification of Adult T-Cell Leukemia/Lymphoma (ATLL), which of the following is a typical immunophenotypic marker?
What is the characteristic morphology of leukemic cells often seen in the peripheral blood of patients with the leukemia phase of Adult T-Cell Leukemia/Lymphoma (ATLL)?
What is the characteristic morphology of leukemic cells often seen in the peripheral blood of patients with the leukemia phase of Adult T-Cell Leukemia/Lymphoma (ATLL)?
What is a distinctive clinical feature commonly associated with the endemic subtype of Burkitt Lymphoma?
What is a distinctive clinical feature commonly associated with the endemic subtype of Burkitt Lymphoma?
Which virus is most strongly associated with the endemic subtype of Burkitt Lymphoma?
Which virus is most strongly associated with the endemic subtype of Burkitt Lymphoma?
What microscopic feature is characteristic of Follicular Lymphoma (FL) cells?
What microscopic feature is characteristic of Follicular Lymphoma (FL) cells?
How do centrocytes and centroblasts differ in Follicular Lymphoma (FL)?
How do centrocytes and centroblasts differ in Follicular Lymphoma (FL)?
What is typically seen in bone marrow biopsies of Follicular Lymphoma (FL)?
What is typically seen in bone marrow biopsies of Follicular Lymphoma (FL)?
A patient with Follicular Lymphoma (FL) is found to have B cells that are IgD+, LCR+, and CD10+. What do these markers indicate?
A patient with Follicular Lymphoma (FL) is found to have B cells that are IgD+, LCR+, and CD10+. What do these markers indicate?
What genetic abnormality is most associated with Mantle Cell Lymphoma (MCL)?
What genetic abnormality is most associated with Mantle Cell Lymphoma (MCL)?
What is a common clinical presentation in patients with Mantle Cell Lymphoma (MCL)?
What is a common clinical presentation in patients with Mantle Cell Lymphoma (MCL)?
What is a key diagnostic feature observed in the peripheral blood smear (PBS) of patients with Mantle Cell Lymphoma (MCL)?
What is a key diagnostic feature observed in the peripheral blood smear (PBS) of patients with Mantle Cell Lymphoma (MCL)?
What is the typical clinical course of Mantle Cell Lymphoma (MCL)?
What is the typical clinical course of Mantle Cell Lymphoma (MCL)?
Which genetic rearrangement is associated with Diffuse Large B-Cell Lymphoma (DLBCL) and is also seen in Follicular Lymphoma?
Which genetic rearrangement is associated with Diffuse Large B-Cell Lymphoma (DLBCL) and is also seen in Follicular Lymphoma?
Which of the following best describes the clinical behavior of Diffuse Large B-Cell Lymphoma (DLBCL)?
Which of the following best describes the clinical behavior of Diffuse Large B-Cell Lymphoma (DLBCL)?
What is a common site of extranodal involvement in Diffuse Large B-Cell Lymphoma (DLBCL)?
What is a common site of extranodal involvement in Diffuse Large B-Cell Lymphoma (DLBCL)?
What is a morphological feature typically observed in DLBCL cells?
What is a morphological feature typically observed in DLBCL cells?
What does the presence of villous lymphocytes typically indicate in Splenic Marginal Zone Lymphoma (SMZL)?
What does the presence of villous lymphocytes typically indicate in Splenic Marginal Zone Lymphoma (SMZL)?
What is a common clinical manifestation of Splenic Marginal Zone Lymphoma (SMZL)?
What is a common clinical manifestation of Splenic Marginal Zone Lymphoma (SMZL)?
Which of the following is characteristic of Mycosis Fungoides (MF) in its early stages?
Which of the following is characteristic of Mycosis Fungoides (MF) in its early stages?
What is the typical morphology of Mycosis Fungoides/Sezary Syndrome (MF/SS) cells?
What is the typical morphology of Mycosis Fungoides/Sezary Syndrome (MF/SS) cells?
What is a diagnostic criterion for Sezary Syndrome?
What is a diagnostic criterion for Sezary Syndrome?
Among the subtypes of Anaplastic Large Cell Lymphoma (ALCL), what is typically associated with a better prognosis?
Among the subtypes of Anaplastic Large Cell Lymphoma (ALCL), what is typically associated with a better prognosis?
Classic Hodgkin's Lymphoma is identified by the presence of what distinctive cell type?
Classic Hodgkin's Lymphoma is identified by the presence of what distinctive cell type?
Flashcards
Myeloproliferative Neoplasm
Myeloproliferative Neoplasm
Clonal hematopoietic disorders caused by genetic mutation in HSCs, resulting in expansion, excessive production and accumulation of mature blood cells.
Philadelphia chromosome
Philadelphia chromosome
A genetic abnormality confirms CML diagnosis involving reciprocal translocation between chromosomes 9 and 22, leading to BCR-ABL1 fusion protein formation.
BCR-ABL1 fusion protein
BCR-ABL1 fusion protein
A fusion protein resulting from the translocation of ABL1 proto-oncogene and BCR. It has tyrosine kinase activity, leading to increased proliferation of myeloid cells.
Chronic Myeloid Leukemia (CML)
Chronic Myeloid Leukemia (CML)
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Leukocytosis with left shift
Leukocytosis with left shift
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Thrombocytosis
Thrombocytosis
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Extramedullary Granulopoiesis
Extramedullary Granulopoiesis
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Leukocyte Alkaline Phosphatase (LAP)
Leukocyte Alkaline Phosphatase (LAP)
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Polycythemia Vera (PV)
Polycythemia Vera (PV)
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JAK2 V617F mutation
JAK2 V617F mutation
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Panmyelosis
Panmyelosis
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Increased PBS in PV
Increased PBS in PV
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BM fibrosis
BM fibrosis
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Blood hyperviscosity
Blood hyperviscosity
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Essential Thrombocythemia (ET)
Essential Thrombocythemia (ET)
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MPL and CALR mutations
MPL and CALR mutations
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Triple Negative
Triple Negative
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Megakaryocyte proliferation
Megakaryocyte proliferation
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Increased reticulin
Increased reticulin
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Primary Myelofibrosis (PMF)
Primary Myelofibrosis (PMF)
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Leukoerythroblastosis
Leukoerythroblastosis
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Intense Fibrosis
Intense Fibrosis
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Dacryocytes
Dacryocytes
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Differentiation Stage
Differentiation Stage
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Dysmegakaryopoeisis
Dysmegakaryopoeisis
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"Dys-" in Myelodysplastic Neoplasms
"Dys-" in Myelodysplastic Neoplasms
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"Myelo-" in Myelodysplastic Neoplasms
"Myelo-" in Myelodysplastic Neoplasms
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"-Poietic" in Myelodysplastic Neoplasms
"-Poietic" in Myelodysplastic Neoplasms
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Myelodysplastic Syndrome/Neoplasm (MDS)
Myelodysplastic Syndrome/Neoplasm (MDS)
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Progressive cytopenias in MDS
Progressive cytopenias in MDS
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Phenotypically normal HSCs in Etiology of MDS
Phenotypically normal HSCs in Etiology of MDS
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CCUS
CCUS
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CHIP
CHIP
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Mutations resulting from therapy
Mutations resulting from therapy
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Inherited mutations
Inherited mutations
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Myeloid Neoplasm Post Cytotoxic Therapy (MN-PCT)
Myeloid Neoplasm Post Cytotoxic Therapy (MN-PCT)
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Dysmyelopoiesis
Dysmyelopoiesis
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WHO Threshold for dysplasia
WHO Threshold for dysplasia
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International Consensus Classification
International Consensus Classification
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Granulocyte Abnormalities
Granulocyte Abnormalities
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MDS with low blasts and isolated 5q deletion (MDS-5q)
MDS with low blasts and isolated 5q deletion (MDS-5q)
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MDS with low blasts and SF3B1 mutation (MDS-SF3B1)
MDS with low blasts and SF3B1 mutation (MDS-SF3B1)
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MDS-LB single lineage dysplasia
MDS-LB single lineage dysplasia
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Childhood MDS with increased blasts (cMDS-IB)
Childhood MDS with increased blasts (cMDS-IB)
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MDS/MPN with SF3B1 mutation and thrombocytosis
MDS/MPN with SF3B1 mutation and thrombocytosis
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Lymphoid Leukemias
Lymphoid Leukemias
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Mature Lymphoid Neoplasms
Mature Lymphoid Neoplasms
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Lymphomas
Lymphomas
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"B" Symptoms
"B" Symptoms
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Immunophenotyping
Immunophenotyping
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Establish Clonality
Establish Clonality
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Molecular Testing
Molecular Testing
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FISH
FISH
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Ann Arbor Staging
Ann Arbor Staging
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Ann Arbor: Key Factors
Ann Arbor: Key Factors
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Lugano Classification
Lugano Classification
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Internal Prognostic Index (IPI)
Internal Prognostic Index (IPI)
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Acute Lymphoblastic Leukemia
Acute Lymphoblastic Leukemia
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T-ALL/T-LBL
T-ALL/T-LBL
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Lymphoblast Morphology
Lymphoblast Morphology
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High Lymphoblast Load
High Lymphoblast Load
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ETV6::RUNX1
ETV6::RUNX1
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IWCLL Criteria
IWCLL Criteria
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Rai/Binet Classification
Rai/Binet Classification
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Heavy Chain IGHV mutation
Heavy Chain IGHV mutation
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Monoclonal B-Cell Lymphocytosis
Monoclonal B-Cell Lymphocytosis
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Small Lymphocytic Lymphoma
Small Lymphocytic Lymphoma
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Adult T-Cell Leukemia/Lymphoma (ATLL)
Adult T-Cell Leukemia/Lymphoma (ATLL)
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Acute ATLL
Acute ATLL
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Leukemia phase of ATLL
Leukemia phase of ATLL
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Burkitt Lymphoma (BL)
Burkitt Lymphoma (BL)
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Endemic Burkitt Lymphoma
Endemic Burkitt Lymphoma
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Leukemic phase BL
Leukemic phase BL
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Follicular Lymphoma (FL)
Follicular Lymphoma (FL)
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FL cell characteristics
FL cell characteristics
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Centrocytes/Centroblasts in FL
Centrocytes/Centroblasts in FL
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Mantle Cell Lymphoma (MCL)
Mantle Cell Lymphoma (MCL)
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Diagnosis of MCL.
Diagnosis of MCL.
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Diffuse Large B Cell Lymphoma (DLBCL)
Diffuse Large B Cell Lymphoma (DLBCL)
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DLBCL cell morphology.
DLBCL cell morphology.
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Marginal Zone Lymphoma. (MZL)
Marginal Zone Lymphoma. (MZL)
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MALT Lymphoma subtype
MALT Lymphoma subtype
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Splenic Marginal Zone Lymphoma (SMZL)
Splenic Marginal Zone Lymphoma (SMZL)
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Mycosis Fungoides/Sezary Syndrome(MF/SS)
Mycosis Fungoides/Sezary Syndrome(MF/SS)
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Diagnosis via topical therapies.
Diagnosis via topical therapies.
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Anaplastic Large Cell Lymphoma (ALCL)
Anaplastic Large Cell Lymphoma (ALCL)
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Classic Hodgkin's Lymphoma Markers
Classic Hodgkin's Lymphoma Markers
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Hodgkin or Non Hodgkin.
Hodgkin or Non Hodgkin.
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Study Notes
Adult T-Cell Leukemia/Lymphoma (ATLL)
- Post-thymic neoplastic disorder of T cells
- Associated with HTLV-1
- Can be transmitted through transplacental route, breastfeeding, blood, and intercourse
- Four clinical subtypes exist
- Acute ATLL
- Aggressive
- Exhibits extensive extranodal involvement of peripheral blood and skin
- Leukemia phase
- Marked by leukocytosis
- Presence of "Flower Cell"
- Positive for CD 3, CD 4, CCR 4, and CD 25
- Negative for CD 7 and CD 8
- Acute ATLL
- Acute subtype has a poor prognosis with a median survival of 8 months
- Chronic subtype has a better prognosis
- Treatments include Interferon-alpha, azidothymidine (AZT), anti-CCR4 antibody (Mogamulizumab), and HSCT
Burkitt Lymphoma (BL)
- Aggressive malignancy of mature B cells
- Characterized by rapid tumor growth
- Three subtypes identified
- Endemic
- Primarily affects children
- Common in Equatorial Africa
- Features extranodal involvement, particularly orbit and mandible
- Positive for EBV genome
- Sporadic
- Common in Western regions
- Often involves abdominal disease
- Bone marrow infiltration present in 70% of cases
- CNS involvement in 1/3 of cases
- Immunodeficiency-associated
- Associated with HIV, post-transplantation, and congenital immunodeficiency
- Primarily affects blood and bone marrow
- Endemic
- Leukemia phase
- B cells features finely clumped chromatin and deeply basophilic cytoplasm with distinct vacuoles
- Bone marrow or lymph node biopsy shows a characteristic "Starry Sky" pattern
- Mature B cell markers
- Positive for IgD, LCR, and CD 10
- Negative for CD 34 and TdT
- Highly responsive to chemotherapy
- Curable in 90% of cases with early-stage disease and 60-80% in advanced cases
- Associated with risk for tumor lysis syndrome
Follicular Lymphoma (FL)
- Neoplastic disorder of germinal B cells
- Second most common NHL
- Typically affects middle-aged to older adults
- Generally considered incurable
- Exhibits painless lymphadenopathy
- FL cells display scant cytoplasm, heavily condensed chromatin, and distinctive deep nuclear clefts at sharp angles
- Bone marrow biopsy shows localization of tumor cells in a paratrabecular distribution
- Includes Centrocytes
- Small in size
- irregular cells with angular appearance
- Centroblasts
- Larger
Grading of Follicular Lymphoma
- Relies on the number of large cells
- Grade 3 indicates a poorer outcome
- Grade 1 and 2: 0-15 centroblasts per high power field (HPF)
- Grade 1: 0-5 centroblasts per HPF
- Grade 2: 6-15 centroblasts per HPF
- Grade 3: >15 centroblasts per HPF
- Grade 3A: Centrocytes are present
- Grade 3B: Centrocytes are absent
- Advanced stage, asymptomatic
- Watchful waiting
- Rituximab
- Advanced stage, symptomatic
- Chemotx
- Chemo-immunotx
- Radio-immunotx
Mantle Cell Lymphoma (MCL)
- Accounts for 6% of Non-Hodgkin’s Lymphoma
- Median age at diagnosis is 68 years
- More common in males
- Generally clinically aggressive
- Characterized by extensive lymphadenopathy and extranodal involvement, especially in the GI tract
- Peripheral blood smear (PBS)
- Lymphoma cells constitute 75% of cells
- Absolute lymphocytosis observed in >50% of cases
- Demonstrates a spectrum of morphologic variants
- Presents with t(11,14), leading to overexpression of cyclin D1
- SOX11 is negative in cyclin D1
Mantle Cell Lymphoma Prognostic Index (MIPI)
- Points are assigned based on age, WBC, LDH, and ECOG PS
- Points 0: Age less than 50, WBC less than 6,700, LDH/ULN LDH Less than 0.67, ECO Status 0-1
- Points 1: Age 50-59, WBC 6,700 – 9,999, LDH/ULN LDH 0.67 – 0.99
- Points 2: Age 60-69, WBC 10, 0 – 14, 999, LDH/ULN LDH 1.00 – 1.49, ECOG PS 2-4
- Points 3: Age 70, WBC 15, 000, LDH/ULN LDH 1.500
- Treatments include chemo-immunotherapy, HSCT and Rituximab
Diffuse Large B Cell Lymphoma (DLBCL)
- Most common form of Non-Hodgkin’s Lymphoma
- May arise de novo or through transformation of a more indolent lymphoma
- Considered aggressive but curable in 2/3 of affected patients
- Rapidly expanding painless lymphadenopathy in one or more sites
- Common extranodal sites include the GI tract, testis, and bone
- Bone marrow involvement may or may not be concordant to node/extranodal site
- DLBCL cells exhibit a leukemic blast appearance
- Pleomorphic, with large/irregular nuclei
- Prominent nucleoli
- Variably clumped chromatin
- Can arise from mutations
- tBCL6 gene - (30%)
- tBCL2 gene, t(14;18) - (20-30%)
- Also seen in Follicular Lymphoma (FL)
- MYC gene rearrangement
- Also seen in Burkitt Lymphoma
- Classified into two subtypes
- Germinal Center DLBCL
- Favors a more favorable prognosis
- Non-germinal Center DLCBL
- Germinal Center DLBCL
Marginal Zone Lymphoma (MZL)
- Indolent B-cell lymphoma
- Associated with chronic antigen stimulation
- Divided into three subtypes
- Extranodal marginal zone lymphoma of MALT
- Splenic marginal zone lymphoma
- Nodal marginal zone lymphoma
- MALT lymphoma
- Most common type of MZL
- Associated with organs lacking lymphoid tissue exposed to chronic inflammation
- Stomach is often involved
- Treatment is often symptomatic
- Splenic Marginal Zone Lymphoma (SMZL)
- Less than 1% of Non-Hodgkin’s Lymphoma
- Mainly affects adults >50 years old
- Characterized by symptoms such as massive splenomegaly and absolute lymphocytosis
- Presence of villous lymphocytes (similar to those in hairy cell leukemia)
- Negative for CD 103 and Annexin A
- Treatment includes watch and wait approach and rituximab
- Nodal Marginal Zone Lymphoma (NMZL)
- Accounts for 1% of Non-Hodgkin’s Lymphoma
Additional NMZL features
- Typically affects adults >50 years old
- NMZL cells extend from the mantle-marginal zone interface
- May be associated with Hepatitis C
Mycosis Fungoides/Sezary Syndrome (MF/SS)
- Cutaneous T-cell lymphoma
- Primarily affects the elderly
- More common in males
- Divided into
- Mycosis Fungoides
- Accounts for 60-70% of cases
- Confined to the skin
- May spread in later stages
- Indolent
- Sezary Syndrome
- Aggressive
- Systemic disorder
- Primarily involves the peripheral blood
- Exhibits worst prognosis
- Mycosis Fungoides
- CD 4+ T cells
- MF/SS cells
- exhibit scant cytoplasm
- Cerebriform, folded nucleus
- Mycosis Fungoides Characteristics
- Psoriatic-like skin lesions
- Localization of atypical cells in Pautrier microabscesses in the epidermis
- Sezary Syndrome Characteristics includes
- Erythroderma
- Generalized lymphadenopathy
- Sezary cells in skin, lymph nodes, and peripheral blood
- Diagnosis
- All three characteristics plus at least one of the following
- Increased CD 4+ T cells: resulting in a CD 4/CD 8 ratio of 10 or greater
- Loss of at least one T cell antigen
- Absolute Sezary cell count >1,000 cells/uL in the peripheral blood
- All three characteristics plus at least one of the following
- Mycosis Fungoides Treatment is symptomatic and included
- Topical steroids
- Nitrogen mustard
- Phototherapy
- Sezary Syndrome has the worst prognosis and is treated with chemotherapy
Anaplastic Large Cell Lymphoma (ALCL)
- T-cell lymphoma presenting with large pleomorphic cells
- Positive for CD 30
- Two main subtypes
- ALK (+)
- Accounts for 10-30% of childhood lymphomas
- Affects younger individuals
- Exhibits better prognosis
- ALK (-)
- Affects older patients (57 years old)
- May be associated with breast implants
- ALK (+)
- Advanced stage of presentation
- B symptoms
- Organ involvement includes lymph nodes, skin, bones, and soft tissue (liver, lungs)
- Treatments include chemotherapy, monoclonal antibodies, and HSCT
Peripheral T-Cell Lymphoma, Not Otherwise Specified (PTCL-NOS)
- T-cell mismatch
- Negative for CD 7 and CD 5
- Exhibits variable clinical course
- Treatments include combination therapy and monoclonal antibodies
Hodgkin’s Lymphoma
- Nodal-based disease
- Rare
- Primarily affects a younger age group
Classic Hodgkin's Lymphoma
- Accounts for 95% of cases
- Exhibits a bimodal age distribution, affecting individuals in their 30s and those after 50 years old
- Characterized by cervical/supraclavicular or mediastinal adenopathy
- Presence of Reed Sternberg Cells, which exhibit an "owl’s eye” appearance
- Positive for CD 30 and CD 15
- Reed Sternberg Cells Seen in lymph nodes
- Identified by 4 subtypes
- Nodular sclerosis
- Lymphocyte-rich
- Mixed cellularity
- Lymphocyte depleted
- Nodular Lymphocyte Predominant Hodgkin Lymphoma (NLPHL)
- Young adults
- Peripheral lymphadenopathy
- Mediastinal adenopathy is uncommon
- Presence of Lymphocytic histiocytic (L&H) cells exhibit
- Large cells with scant cytoplasm
- Folded single nucleus
- “Popcorn cells”
- Negative for CD 15 and CD 30
- Positive for epithelial membrane antigen (EMA) – 50%
- Hodgkin's Lymphoma Treatment is the combination of
- Combination therapy
- Radiotherapy
- Alkylating agents
- HSCT
- Immune checkpoint blockade inhibitors
Hodgkin Lymphoma vs Non-Hodgkin Lymphoma
- Hodgkin Lymphoma
- Spreads contiguously
- Rare involvement of extranodal sites
- Contain "Reed-Sternberg Cells"
- Non-Hodgkin Lymphoma
- Can spread non-contiguously
- Involves extranodal sites, including skin, brain, and GI tract
- Lacks Reed-Sternberg Cells
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