Medicine Marrow Pg 131-140 (Hematology)
50 Questions
0 Views

Medicine Marrow Pg 131-140 (Hematology)

Created by
@ArdentHouston

Podcast Beta

Play an AI-generated podcast conversation about this lesson

Questions and Answers

What is the most common childhood cancer?

  • B cell ALL (correct)
  • T cell Lymphoma
  • Chronic Lymphocytic Leukemia (CLL)
  • Acute Myeloid Leukemia (AML)

    • Fever occurs in 50% of cases of B cell ALL.

    True

    What is the primary antigen associated with immature B cells?

    CALLA antigen (CD 10⁺)

    The classical presentation of T cell ALL includes ______ and pleural effusion.

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

    Match the type of leukemia with its characteristic feature:

    <p>B cell ALL = Features of bone marrow failure T cell ALL = Mediastinal mass + pleural effusion Acute Myeloid Leukemia (AML) = No fever HTLV-1 = Adult T cell lymphoma</p> Signup and view all the answers

    Which of the following is a risk factor for both Acute Myeloid Leukemia and Acute Lymphoblastic Leukemia?

    <p>Down syndrome</p> Signup and view all the answers

    Adults with Acute Lymphoblastic Leukemia typically present with anemia, neutropenia, and thrombocytopenia.

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

    What is a common presenting feature in children with Acute Lymphoblastic Leukemia?

    <p>Limping, bone tenderness, inability to walk</p> Signup and view all the answers

    In children with Acute Lymphoblastic Leukemia, a high count of ______ is associated with a very poor prognosis.

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

    Match the following laboratory features with their potential implications:

    <p>Anemia = Low red blood cell count Thrombocytopenia = Increased risk of bleeding High leukocyte count = Potential sign of leukemia Hypereosinophilia = Precedes presence of blasts</p> Signup and view all the answers

    What is an indication for Allogenic Hematopoietic Stem Cell Transplantation (AHSCT)?

    <p>MRD (-)</p> Signup and view all the answers

    High-dose Cytarabine is mandatory for patients with standard risk and MRD (-).

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

    What is the prognosis for a patient with Acute Promyelocytic Leukemia (AML M3) when treated?

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

    A patient with 0.01% blasts in marrow is considered MRD ______.

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

    Match the following drugs with their categories:

    <p>Azacitidine = Hypomethylating agent Ivosidenib = Isocitrate dehydrogenase inhibitor Gemtuzumab-Ozogamicin = Anti-CD33 Decitabine = Hypomethylating agent</p> Signup and view all the answers

    What is a characteristic feature of hypergranular promyelocytes?

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

    Acute leukemia can lead to early mortality due to severe hemorrhage.

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

    What treatment should be initiated early if acute myeloid leukemia M3 is suspected?

    <p>All-trans retinoic acid (ATRA)</p> Signup and view all the answers

    In patients with high-risk acute leukemia, the WBC count is greater than ______.

    <p>10,000</p> Signup and view all the answers

    Match the following types of promyelocytes with their characteristics:

    <p>Hypergranular = Dark, large, dumbbell-shaped nucleus Hypogranular = Low incidence</p> Signup and view all the answers

    Which marker is NOT associated with B cells?

    <p>CD 3</p> Signup and view all the answers

    Naive B cells do not express CD 19.

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

    What can failure of apoptosis lead to in lymphoid cells?

    <p>Clonal proliferation</p> Signup and view all the answers

    The preferred marker for identifying precursor B cell ALL is _____ .

    <p>not provided</p> Signup and view all the answers

    Match the B-cell types with their corresponding prognosis:

    <p>pro-B cell = Bad Pre-B cell = Good Immature B cell = Bad Naive B cell = N/A in this context</p> Signup and view all the answers

    Which of the following is considered a bad prognostic factor for Acute Leukemia?

    <p>High presenting leukocyte count</p> Signup and view all the answers

    Therapy-associated AML is considered a good prognostic factor for Acute Leukemia.

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

    What age group is considered a bad prognostic factor in Acute Leukemia?

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

    Patients experiencing anemia, leukopenia, or thrombocytopenia for more than one month before diagnosis are considered to have __________ prognostic factors for Acute Leukemia.

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

    Match the following treatments or factors with their classification:

    <p>t(8; 21) = Standard Risk t(16; 16) = Standard Risk Age (elderly) = Bad Prognostic Factor MDS-associated AML = Bad Prognostic Factor</p> Signup and view all the answers

    What is the survival rate for children under 10 years old diagnosed with Acute Lymphoblastic Leukemia?

    <p>90-95%</p> Signup and view all the answers

    Acute Myeloid Leukemia is characterized by the presence of nucleoli in its cells.

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

    What type of leukocytes primarily affect Acute Lymphoblastic Leukemia?

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

    Acute Myeloid Leukemia (AML) is associated with the presence of ________, which are absent in Acute Lymphoblastic Leukemia (ALL).

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

    Match the features with the corresponding type of cell (Lymphoblast/Myeloblast):

    <p>Smaller = Lymphoblast Present = Myeloblast Negative = Lymphoblast Positive = Myeloblast</p> Signup and view all the answers

    What is the prognosis associated with the t(8; 21) genetic abnormality?

    <p>Good prognosis</p> Signup and view all the answers

    The presence of an NPM mutation is associated with a bad prognosis.

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

    Which CD markers are associated with myeloblasts?

    <p>CD11b, CD13, CD33, CD117, HLA-DR</p> Signup and view all the answers

    The genetic abnormality t(9; 22) is associated with a ______ prognosis.

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

    Match the following genetic abnormalities with their associated characteristics:

    <p>t(8; 21) = Common in children with good prognosis t(16; 16) = Inversion related to myelomonocytic leukemia t(9; 22) = Associated with bad prognosis c-kit mutation = Present in t(8; 21) or t(16; 16) with bad prognosis</p> Signup and view all the answers

    Which of the following factors is associated with a bad prognosis in Acute Lymphoblastic Leukemia?

    <p>The patient has a mediastinal mass</p> Signup and view all the answers

    Transplantation is recommended only after a positive result in the bone marrow study for ALL.

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

    What is the goal of the induction phase in the treatment of Acute Lymphoblastic Leukemia?

    <p>To achieve remission</p> Signup and view all the answers

    In the treatment regimen for Acute Lymphoblastic Leukemia, __________ is used to reduce CNS relapses.

    <p>intrathecal methotrexate</p> Signup and view all the answers

    Match the following features with their corresponding prognosis type in Acute Lymphoblastic Leukemia:

    <p>Race: White = Good prognosis CNS involvement: + = Bad prognosis Type: LI = Good prognosis Age: 10 years = Bad prognosis</p> Signup and view all the answers

    Which of the following skin involvement characteristics is true for leukemia cutis?

    <p>Non tender, violaceous nodular skin lesions</p> Signup and view all the answers

    Myeloblasts are characterized by the presence of prominent nucleoli.

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

    What type of anemia is commonly seen in patients with acute leukemia?

    <p>Normocytic normochromic anemia</p> Signup and view all the answers

    In acute leukemia, the total WBC count is typically less than ______ cells.

    <p>4000</p> Signup and view all the answers

    Match the following features with their corresponding type of cell:

    <p>Myeloblast = 1-4 prominent nucleoli Promyelocyte = Clustering of Auer rods (faggot cells)</p> Signup and view all the answers

    Study Notes

    B Cell Types

    • Pro B cell: Incomplete rearrangement of heavy chain
    • Pre B cell: Complete rearrangement of heavy chain, CD 10⁺ (CALLA antigen), good prognosis
    • Immature B cell: Complete rearrangement of heavy and light chain, loses CD 10, bad prognosis

    Acute Lymphocytic Leukemia (ALL)

    • Most common childhood cancer
    • Features of bone marrow failure: fever, cytokine production by lymphoblasts, marrow cavity expansion, bleeding tendencies, CNS involvement
    • B cell ALL: Most common subtype in childhood
    • T cell ALL: Often presents with mediastinal mass and pleural effusion
    • CNS involvement is common in both types

    Acute Myeloid Leukemia (AML)

    • No fever
    • HTLV-1: Organism that produces adult T cell lymphoma
    • AML M3: Presents with bleeding and pancytopenia, has good prognosis with treatment
    • Granules in promyelocytes activate the extrinsic cascade, leading to DIC and hemorrhage
    • Prognosis is influenced by age, leukocyte count, anemia, thrombocytopenia, and genetic factors
    • Treatment includes induction, consolidation, and maintenance phases
    • Drugs: hypomethylating agents (azacitidine, decitabine), Venetoclax, Gemtuzumab-Ozogamicin, Ivosidenib

    Risk Factors:

    • Down syndrome
    • Malignancy: Transient abnormal myelopoiesis
    • Defective DNA repair: Ataxia-telangiectasia, Bloom's syndrome
    • Radiation

    Presenting Features

    • Adults: Pancytopenia
    • Children: Limping, bone tenderness, inability to walk, dyspnea, mediastinal widening
    • T cell ALL in adolescents: High count ALL = poor prognosis, involves sanctuary sites (CNS, testes)
    • Relapse after treatment: Requires intrathecal chemotherapy
    • Rare symptoms: Pallor, petechiae, ecchymosis, fatigue, lethargy, organomegaly

    Laboratory Features

    • In blood: Anemia, neutropenia, thrombocytopenia, circulating blasts, variable counts, hypereosinophilia preceding blasts
    • In CSF: Leukemic blasts

    Evaluation After Induction Phase

    • 5% blasts in marrow: Refractory AML, inevitable death
    • MRD: Predicts risk of relapse (0.01% blasts = bad prognosis)

    Consolidation Phase

    • Allogenic Hematopoietic Stem Cell Transplantation (AHSCT): Indicated for patients with MRD (-)
    • High-dose Cytarabine: Indicated in standard risk with MRD (-), transplant not mandatory

    Lymphoma

    Diagram

    • Common lymphoid progenitor gives rise to B cells, T cells, and NK cells
    • CD 19: B cell marker, CD 3: T cell marker, CD 56: NK cell marker

    B Cell Lymphoma

    • Pro B cell: Incomplete rearrangement of heavy chain, bad prognosis
    • Pre B cell: Complete rearrangement of heavy chain, good prognosis, loses CD 34 and HLA-DR
    • Immature B cell: Complete rearrangement of heavy and light chain, loses CD 10, bad prognosis
    • Naive B cell: No antigen encounter
    • Precursor B cell ALL:

    Pathophysiology (ALL & Myeloblast)

    • ALL: Better prognosis in children, affects bone marrow, can involve thymus (mediastinal widening and dyspnea)
    • Myeloblast: Poor prognosis, affects bone marrow, can involve thymus (mediastinal widening and dyspnea)

    Lymphoblast vs Myeloblast

    • Lymphoblast: Smaller, absent nucleoli, absent granules
    • Myeloblast: Larger, present nucleoli, present granules

    Prognostic Factors (ALL)

    • Good prognosis: White race, 2-9 years, female, no CNS involvement, no hepatosplenomegaly/lymphadenopathy/mediastinal mass, LI type, hyperdiploidy (t(12;21)), NPM mutation without FLT3-ITD, CEBPA mutation
    • Bad prognosis: Black race, >10 years, male, CNS involvement, hepatosplenomegaly/lymphadenopathy/mediastinal mass, L2/L3 type, hypodiploidy (t(9;22), t(4; 11), t(1;19)), FLT3-ITD mutation

    Treatment of ALL

    • Induction Phase (2 cycles): Goal is remission, BFM-19 protocol (steroids, vincristine, daunorubicin, cyclophosphamide), bone marrow study for MRD status
    • Negative MRD: Consolidation phase (2 more cycles of chemotherapy)
    • Positive MRD: 2 cycles of chemotherapy, then transplant
    • Maintenance Phase: Methotrexate + 6-mercaptopurine for 2-3 years
    • Other Drugs: Rituximab (CD20+ve), intrathecal methotrexate, blinatumomab (bispecific T-cell engager), tyrosine kinase inhibitors (BCR-ABL+ve)

    Cytology

    • MPO and Sudan black +: M4, M5, M7
    • NSE, PAS +: M6, M7

    Immunophenotyping

    • Myeloblast: CDIIb, CD13, CD33, CD117, HLA-DR
    • Promyelocyte: CD13, CD33
    • Myelomonoblast: CDIIb, CD13, CD33, CD14, HLA-DR
    • Monoblast: CDIIb, CD13, CD33, CDIIC, HLA-DR
    • Erythroid leukemia: Glycophorin, spectrin
    • Megakaryocytic (M7): CD41, CD61

    Cytogenetics & Molecular Genetics

    • Recurrent genetic abnormalities:
      • t(8; 21): Most common, RUNX1-RUNXT translocation, good prognosis
      • t(16; 16): Abnormal eosinophils, myelomonocytic leukemia, good prognosis
      • t(9; 22): Bad prognosis (except in CML)
    • Mutations:
      • c-kit mutation: Bad prognosis
      • NPM (Nucleophosmin-1) mutation: Cup-shape blasts, good prognosis, most common
      • CEBPA mutation: Good prognosis

    Prognosis (European Risk Stratification)

    • Favourable: t(8; 21), t(16; 16), NPM mutation without FLT3-ITD, CEBPA mutation
    • Intermediate: No cytogenetic abnormalities
    • Unfavourable: All other mutations

    Skin Involvement

    • Leukemia cutis: Non-tender, violaceous nodular skin lesions
    • Sweet syndrome: Tender, erythematous, violaceous papules/plaques (Neutrophilic infiltration of dermis), site is face and neck
    • Causes: AML (monoblastic lineage), G-CSF therapy, PMF
    • Treatment: Chemotherapy, steroids

    Evaluation (Skin Involvement)

    • Blood picture: Normocytic normochromic anemia, low platelets (<1 lac cells), low WBC count (<4000 cells)

    Morphology (Myeloblast & Promyelocyte)

    • Myeloblast: Large, uniform blast cell with high nuclear:cytoplasm ratio, pale blue scanty cytoplasm and granules, 1-4 prominent nucleoli, Auer rods present (more prominent in AML M3), fine dispersed chromatin
    • Promyelocyte: Larger cell, more visible granules, absence of nucleoli, clustering of Auer rods (faggot cells), AML M3 (promyelocytic leukemia)

    Studying That Suits You

    Use AI to generate personalized quizzes and flashcards to suit your learning preferences.

    Quiz Team

    Description

    Test your knowledge on B cell types and acute leukemias, including their characteristics, symptoms, and prognostic factors. This quiz covers both Acute Lymphocytic Leukemia (ALL) and Acute Myeloid Leukemia (AML), focusing on their presentations and implications in childhood cancer.

    More Like This

    Use Quizgecko on...
    Browser
    Open
    Browser
    Browser