HEMA 2 FINAL EXAM
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What is the primary function of synthetic antibodies in the identification of cells?

  • To stimulate the production of hybridoma cells
  • To break down the cytoplasm of cancerous cells
  • To differentiate between cancerous and non-cancerous cells
  • To detect antigens or CD markers (correct)

    • What is the distinguishing characteristic of lymphoblasts in ALL L1?

  • Large and heterogeneous in size
  • Clefted nuclei and basophilic cytoplasm
  • Abundant cytoplasm and prominent nucleoli
  • Small and homogeneous in size (correct)

    • What is the prognosis for patients with ALL L3?

  • Good prognosis
  • Variable prognosis
  • Poor prognosis (correct)
  • Unknown prognosis

    • What is the characteristic of blasts in M1 acute myeloid leukemia?

    <p>More than 30% blasts</p> Signup and view all the answers

    What is the primary difference between M3 and M4 acute myeloid leukemia?

    <p>Presence of promyelocytes</p> Signup and view all the answers

    What is the percentage of blast cells in peripheral blood and bone marrow to classify leukemia according to the World Health Organization?

    <p>20%</p> Signup and view all the answers

    What is the primary focus of cytogenetic analysis?

    <p>Detecting abnormalities in chromosome number or structure</p> Signup and view all the answers

    What is the characteristic of M5B acute monocytic leukemia?

    <p>More than 80% monocytic cells</p> Signup and view all the answers

    What is the purpose of cytochemistry in diagnosing leukemia?

    <p>To identify cellular enzymes and other chemicals</p> Signup and view all the answers

    What is the purpose of flow cytometry in cell identification?

    <p>To detect antigens or CD markers</p> Signup and view all the answers

    What is the term for the process of analyzing chromosomes to identify abnormalities?

    <p>Karyotyping</p> Signup and view all the answers

    What is the characteristic of lymphoblasts in ALL L2?

    <p>Large and heterogeneous in size</p> Signup and view all the answers

    What is the typical age range for ALL L1?

    <p>Children only</p> Signup and view all the answers

    What is the primary method used to identify cells based on their surface antigens?

    <p>Immunophenotyping (Flow cytometry)</p> Signup and view all the answers

    What is the technique used to produce synthetic antibodies?

    <p>Hybridoma technology</p> Signup and view all the answers

    What is the term for the study of the structure, function, and abnormalities of chromosomes?

    <p>Cytogenetics</p> Signup and view all the answers

    What is the primary application of immunophenotyping in diagnosing leukemia?

    <p>Determining the presence of antigens on cell surfaces</p> Signup and view all the answers

    According to the World Health Organization, what is the primary method used to classify leukemia?

    <p>Combination of multiple methods</p> Signup and view all the answers

    What is the term for the process of using specialized stains to detect cellular enzymes and other chemicals?

    <p>Cytochemistry</p> Signup and view all the answers

    What is the primary focus of cellular morphology in diagnosing leukemia?

    <p>Classifying leukemia based on cell shape and structure</p> Signup and view all the answers

    What is the primary location where myeloma forms tumors?

    <p>Bone marrow</p> Signup and view all the answers

    What is the term for the abnormal, uncontrolled proliferation and accumulation of hematopoietic cells?

    <p>Leukemia</p> Signup and view all the answers

    What is the main difference between acute and chronic leukemia?

    <p>Duration of the disease</p> Signup and view all the answers

    What is the primary cause of fatigue and weakness in leukemia patients?

    <p>Anemia</p> Signup and view all the answers

    What is the name of the person who first recognized leukemia as a distinct clinical disorder?

    <p>Virchow</p> Signup and view all the answers

    What is the main difference between lymphoma and myeloma?

    <p>Type of cells affected</p> Signup and view all the answers

    What is the term for the swelling of lymph nodes?

    <p>Lymphadenopathy</p> Signup and view all the answers

    What is the most common symptom of leukemia?

    <p>Fatigue and weakness</p> Signup and view all the answers

    What is the term for the overcrowding of healthy cells in the bone marrow?

    <p>Crowding out</p> Signup and view all the answers

    What is the primary difference between Hodgkin lymphoma and non-Hodgkin lymphoma?

    <p>Not specified in the text</p> Signup and view all the answers

    What is the characteristic of the cytoplasmic projections in hairy cell leukemia?

    <p>Fine and hair-like</p> Signup and view all the answers

    Which type of lymphoma is characterized by a 'starry sky' pattern?

    <p>Burkitt lymphoma</p> Signup and view all the answers

    What is the primary location where mantle cell lymphoma affects the B-cells?

    <p>Lymph nodes</p> Signup and view all the answers

    What is the characteristic of the nuclei in Sezary cells?

    <p>Irregular and cerebriform</p> Signup and view all the answers

    Which type of lymphoma is considered fast-growing compared to other lymphomas?

    <p>Mantle cell lymphoma</p> Signup and view all the answers

    What is the characteristic of the cytoplasm in Burkitt lymphoma?

    <p>Basophilic and vacuolated</p> Signup and view all the answers

    What is the primary origin of follicular lymphoma?

    <p>Germinal center B cells</p> Signup and view all the answers

    What is the characteristic of the lymphoid cells in mantle cell lymphoma?

    <p>Medium-sized and irregular</p> Signup and view all the answers

    What is the most common type of cutaneous lymphoma?

    <p>Mycosis fungoides</p> Signup and view all the answers

    What is the characteristic of the acid phosphatase reaction in hairy cell leukemia?

    <p>Strong and not inhibited by tartrate</p> Signup and view all the answers

    What is the main reason for a patient's ruddy skin coloration?

    <p>Increase in RBC concentration and viscosity of the blood</p> Signup and view all the answers

    What is the primary effect of diminished plasma volume on RBC mass?

    <p>Relative increase in RBC mass</p> Signup and view all the answers

    What is the primary difference between primary absolute polycythemia and secondary polycythemia with appropriately increased EPO?

    <p>Underlying cause</p> Signup and view all the answers

    What is the characteristic of genetic polycythemia?

    <p>Increased RBC mass and hematocrit</p> Signup and view all the answers

    What is the primary distinction between relative polycythemia and absolute polycythemia?

    <p>RBC mass</p> Signup and view all the answers

    What is the underlying cause of secondary polycythemia with inappropriately increased EPO?

    <p>Neoplasms</p> Signup and view all the answers

    What is the characteristic of primary absolute polycythemia?

    <p>Increased RBC mass and hematocrit</p> Signup and view all the answers

    What is the primary effect of stress on RBC mass?

    <p>Relative increase in RBC mass</p> Signup and view all the answers

    What is the primary distinction between secondary polycythemia with appropriately increased EPO and secondary polycythemia with inappropriately increased EPO?

    <p>EPO levels</p> Signup and view all the answers

    What is the primary characteristic of spurious polycythemia?

    <p>Relative increase in RBC mass</p> Signup and view all the answers

    What is the primary function of the Janus kinase 2 protein?

    <p>Regulation of blood cell growth and division</p> Signup and view all the answers

    What is the most common mutation associated with Myeloproliferative Neoplasms?

    <p>JAK2V617F</p> Signup and view all the answers

    What is the characteristic of Chronic Myelogenous Leukemia?

    <p>Stem cell disorder affecting the granulocytic, monocytic, erythrocytic, and megakaryocytic cell lines</p> Signup and view all the answers

    What is the name of the chromosomal abnormality associated with Chronic Myelogenous Leukemia?

    <p>Philadelphia chromosome</p> Signup and view all the answers

    What is the primary characteristic of Myelofibrosis with Myeloid Metaplasia?

    <p>All of the above</p> Signup and view all the answers

    What is the primary characteristic of Essential Thrombocythemia?

    <p>Thrombocytosis with hemorrhage and platelet dysfunction</p> Signup and view all the answers

    What is the primary characteristic of Polycythemia Vera?

    <p>Absolute increase in red blood cells, white blood cells, and platelets</p> Signup and view all the answers

    What is the frequency of the JAK2V617F mutation in Primary Myelofibrosis?

    <p>65%</p> Signup and view all the answers

    What is the primary characteristic of Chronic Neutrophilic Leukemia?

    <p>Uncontrolled proliferation of neutrophilic cells</p> Signup and view all the answers

    What is the primary focus of cytogenetic analysis in Myeloproliferative Neoplasms?

    <p>Identification of genetic abnormalities</p> Signup and view all the answers

    Study Notes

    Leukemia

    • Leukemia is a disease characterized by the abnormal, uncontrolled proliferation and accumulation of one or more of the hematopoietic cells.
    • It is a malignancy of leukocytes in the blood and bone marrow.

    Symptoms of Leukemia

    • Fatigue and weakness
    • Increased infections
    • Bleeding and bruising
    • Anemia
    • Swollen lymph nodes
    • Enlarged spleen or liver
    • Bone pain or tenderness

    Classification of Leukemia

    • Based on duration: acute (days to 6 months), subacute (2 to 6 months), and chronic (1 or 2 years or more)
    • Based on the number of white blood cells (WBCs) present in peripheral blood: acute leukemia (>30% blast in peripheral blood and bone marrow)

    World Health Organization (WHO) Classification

    • Defines acute leukemia as >20% peripheral blood and bone marrow blasts
    • Subdivides leukemia based on: cellular morphology, cytochemical stains, immunophenotyping, cytogenetic abnormalities, and clinical syndrome

    Cytochemical Analysis

    • Uses specialized stains to detect cellular enzymes and other chemicals in peripheral blood films and bone marrow aspirate smears
    • Helps differentiate hematologic diseases, especially leukemias

    Immunophenotyping (Flow Cytometry)

    • Identifies cells based on the types of markers or antigens present on the cell's surface, nucleus, or cytoplasm
    • Helps identify the lineage of cells using antibodies that detect markers or antigens on the cells

    Acute Lymphoblastic Leukemia (ALL)

    • Subdivided into three categories based on the FAB classification:
      • ALL L1: lymphoblasts are small and homogenous, with scanty cytoplasm and inconspicuous nucleoli
      • ALL L2: lymphoblasts are large and heterogenous, with abundant cytoplasm and nucleoli
      • ALL L3 (Burkitt-Type): lymphoblasts are large, homogenous, and vacuolated, with rarest subclass and poor prognosis

    Acute Myeloid Leukemia (AML)

    • Subdivided into five categories based on the FAB classification:
      • MO: acute myeloid leukemia, minimally differentiated
      • M1: acute myeloid leukemia without maturation
      • M2: acute myeloid leukemia with maturation
      • M3: acute promyelocytic leukemia
      • M4: acute myelomonocytic leukemia
      • M5B: acute monoblastic leukemia with maturation

    Lymphoproliferative Disorders

    • Hairy cell leukemia: a type of lymphoproliferative disorder characterized by small B lymphocytes with abundant cytoplasm and fine (“hairy”) cytoplasmic projections.
    • Bone marrow produces too many abnormal B-lymphocytes, more common in males than females.
    • Cytochemical features: strong acid phosphatase reaction, not inhibited by tartaric acid or tartrate-resistant acid phosphatase (TRAP) stain.

    Mantle Cell Lymphoma

    • Affects the B-cells within the lymph nodes, particularly the "mantle zone" - the outer area surrounding the center.
    • A subtype of non-Hodgkin lymphoma (NHL), considered fast-growing compared to other lymphomas.
    • Originates from B-cells in the lymph nodes.
    • Characterized by medium-sized lymphoid cells with irregular nuclear outlines, derived from the follicular mantle zone.

    Follicular Lymphoma

    • Originates from germinal center B cells and recapitulates follicular architecture in most cases.
    • Numerous closely spaced follicles replace the normal nodal architecture.

    Burkitt Lymphoma

    • Characterized by medium-sized, highly proliferating lymphoid cells with basophilic vacuolated cytoplasm.
    • Lymphoid proliferation is diffuse, with a prominent “starry sky” pattern at low magnification.
    • WHO classification lists three variants: endemic (occurring predominantly in Africa), sporadic, and immunodeficiency-associated.

    Mycosis Fungoides and Sézary Syndrome

    • Mycosis fungoides: the most common cutaneous lymphoma, affecting T lymphocytes.
    • Sézary cells: small to medium-sized lymphoid cells with irregular nuclear outlines (cerebriform nuclei).

    Myeloproliferative Disorders (MPDs)

    • Interrelated clonal hematopoietic stem cell disorders characterized by excessive proliferation of one or more mature myeloid cell lines (e.g., granulocytes, erythrocytes, megakaryocytes, or mast cells)

    Janus Kinase 2 (JAK2)

    • A gene that provides instructions for making a protein involved in cellular signaling pathways that regulate blood cell growth and division in the bone marrow
    • JAK2V617F mutation leads to the production of an abnormal JAK2 protein, promoting uncontrolled growth and division of blood cells

    Myeloproliferative Neoplasms (MPNs)

    • Essential Thrombocythemia (ET)
    • Polycythemia Vera (PV)
    • Idiopathic Myelofibrosis (IMF)
    • Chronic Myelogenous Leukemia (CML)
    • Chronic Neutrophilic Leukemia (CNL)
    • Varies Nucleated Erythrocytes/Megakaryocytes in Myeloproliferation (VNEL)

    Chronic Myelogenous Leukemia (CML)

    • Stem cell disorder affecting granulocytic, monocytic, erythrocytic, and megakaryocytic cell lines
    • Characterized by the presence of the Philadelphia chromosome (90% of cases) and BCR/ABL1 abnormality
    • Patients without the Philadelphia chromosome usually have a poorer prognosis

    Myelofibrosis with Myeloid Metaplasia (Primary Myelofibrosis/Agnogenic Myelofibrosis)

    • Characterized by fibrosis and granulocytic hyperplasia of the bone marrow, with granulocytic and megakaryocytic proliferation in the liver and spleen
    • Associated with splenomegaly, ineffective hematopoiesis, and marrow hypercellularity
    • JAK2 V617F mutation is involved in the pathogenesis (65% of PMF patients) and presence of Dacryocytes

    Essential Thrombocythemia (ET)

    • Chronic MPD characterized by thrombocytosis (>1000x109/L) with spontaneous aggregation of functionally abnormal platelets
    • JAK2 V617F mutation is found in 50-60% of ET patients, supporting the diagnosis of ET
    • Clinical manifestations include hemorrhage, platelet dysfunction, and thrombosis

    Polycythemia Vera (PV)

    • Characterized by an absolute increase in RBC, WBC, and platelets
    • JAK2 V617F mutation is detected in 90-97% of patients with PV
    • Also known as primary absolute polycythemia or polycythemia Rubra vera
    • Patients exhibit a ruddy skin coloration due to increased RBC concentration and blood viscosity
    • Increase RBC mass, decreased EPO, and increased RBC, WBC, and platelet count

    Pathophysiological Classification of Polycythemia

    • Relative polycythemia: normal RBC mass, increased hematocrit, normal EPO, usually due to dehydration, diarrhea, burns, or shock
    • Absolute polycythemia: primary absolute polycythemia (PV), secondary polycythemia with appropriately increased EPO (e.g., hypoxia, high altitudes), and genetic polycythemia (e.g., primary familial congenital polycythemia, Chuvash polycythemia)

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    Related Documents

    Week 12: Malignant Leukocyte Disorders - PDF
    Week 13 Lymphoma Types PDF
    Week 14 Myeloproliferative Disorders/Neoplasms PDF

    Description

    This quiz covers the definition, symptoms, and characteristics of leukemia, including its history, causes, and effects on the body. It also explores leukocyte disorders and their impact on health.

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