B-ALL and Myelofibrosis Quiz
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Questions and Answers

Which mutation is commonly associated with Myelofibrosis?

  • BRAF
  • TP53
  • MPL or CALR (correct)
  • JAK2

    • What presents as tear drop cells in the context of hematological disorders?

  • Normal red blood cells
  • Target cells
  • Dacrocytes (correct)
  • Spherocytes

    • Which of the following indicators is associated with a poor prognosis in patients with hyperdiploidy?

  • B cell lineage establishment
  • Hypodiploidy (correct)
  • CD34 positivity
  • Hyperdiploidy

    • What is the role of TdT in B cell Acute Lymphoblastic Leukemia (B-ALL)?

    <p>Establishing lymphoid lineage</p> Signup and view all the answers

    Which treatment option can be used for relapse in B cell Acute Lymphoblastic Leukemia (B-ALL)?

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

    Which clinical findings are most commonly associated with Myelodysplastic Syndrome (MDS)?

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

    What characterizes the bone marrow findings in Myelodysplastic Syndrome?

    <p>Hypercellular with dysplastic features and clonal populations</p> Signup and view all the answers

    Which chromosomal abnormalities are associated with a good prognosis in MDS?

    <p>del(5q) and del(20q)</p> Signup and view all the answers

    What is a primary characteristic of dysplastic maturation in MDS?

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

    In Myelodysplastic Syndrome, what often leads to the progression to acute myeloid leukemia (AML)?

    <p>Defective differentiation of hematopoietic cells</p> Signup and view all the answers

    What is the average age of diagnosis for patients with Myelodysplastic Syndrome?

    <p>About 60 years old</p> Signup and view all the answers

    Which of the following is a common cytopenia seen in patients with Myelodysplastic Syndrome?

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

    What treatment option may be utilized for eligible patients with Myelodysplastic Syndrome?

    <p>Allogenic stem cell transplant</p> Signup and view all the answers

    What is the relationship between azacitidine and decitabine in the treatment of Acute Myeloid Leukemia?

    <p>They are utilized as outpatient treatments in older, frail patients.</p> Signup and view all the answers

    What laboratory findings are commonly associated with neutropenia in patients with Acute Myeloid Leukemia?

    <p>Low red blood cells and low platelet counts.</p> Signup and view all the answers

    What is a hallmark cytogenetic finding in acute promyelocytic leukemia?

    <p>t(15;17) (q22;11-12)</p> Signup and view all the answers

    Which of the following is NOT a common side effect of azacitidine and decitabine treatment?

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

    In patients with acute promyelocytic leukemia, what is the effect of starting ATRA (all-trans retinoic acid) therapy early?

    <p>It encourages the differentiation of promyelocytes.</p> Signup and view all the answers

    What is one of the poor risk features of myeloid lineage in Acute Myeloid Leukemia?

    <p>Monocytic characteristics</p> Signup and view all the answers

    What complication often associated with neutropenia in patients with Acute Myeloid Leukemia can lead to significant bleeding issues?

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

    What does the presence of large azurophilic cytoplasmic granules indicate in the context of Acute Myeloid Leukemia?

    <p>Immaturity in cell development</p> Signup and view all the answers

    What is the effect of heparin on coagulation factors?

    <p>It inactivates thrombin and activated factor X.</p> Signup and view all the answers

    Which coagulation factors are affected by vitamin K deficiency?

    <p>Factor II, VII, IX, and X.</p> Signup and view all the answers

    What is the primary use of protamine sulfate?

    <p>To neutralize the effects of heparin.</p> Signup and view all the answers

    What laboratory findings are typically associated with vitamin K deficiency?

    <p>Prolonged PT and possibly prolonged PTT.</p> Signup and view all the answers

    What condition could potentially lead to pathologic, systemic activation of hemostatic mechanisms?

    <p>Sepsis (gram negative).</p> Signup and view all the answers

    Which of the following is NOT a result of Warfarin usage?

    <p>Increased platelet aggregation.</p> Signup and view all the answers

    What is the consequence of decreased activity of antithrombin III (AT3)?

    <p>Enhanced coagulation and increased risk of thrombosis.</p> Signup and view all the answers

    What is primarily affected in platelet coagulation with heparin usage?

    <p>Activated factor X.</p> Signup and view all the answers

    Which of the following statements best describes the effect of vitamin K on coagulation?

    <p>It is essential for the synthesis of certain clotting factors.</p> Signup and view all the answers

    What is the outcome of impaired platelet coagulation in the context of prolonged PT?

    <p>Increased risk of bleeding.</p> Signup and view all the answers

    What is the age range with a moderate association of EBV for the occurrence of the mentioned conditions?

    <p>15-35 and &gt;55</p> Signup and view all the answers

    Which treatment is considered for patients with limited-stage disease?

    <p>3 cycles of combo treatment</p> Signup and view all the answers

    Which cell type is specifically mentioned as a characteristic of the condition?

    <p>Reed-Sternberg cells</p> Signup and view all the answers

    What is the best prognosis subtype mentioned?

    <p>Lymphocyte-rich</p> Signup and view all the answers

    Which adverse effect is particularly associated with Bleomycin treatment?

    <p>Pulmonary toxicity</p> Signup and view all the answers

    What condition is characterized by B symptoms including drenching night sweats?

    <p>Hodgkin's lymphoma</p> Signup and view all the answers

    What is the treatment protocol for advanced-stage disease?

    <p>6 cycles of AVD</p> Signup and view all the answers

    Which screening should be performed prior to starting treatment with Bleomycin?

    <p>Pulmonary function tests (PFTs)</p> Signup and view all the answers

    Which characteristic is associated with diffuse large B-cell lymphoma?

    <p>It arises from germinal center B cells.</p> Signup and view all the answers

    What adverse effect is most commonly associated with Doxorubicin?

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

    Which immunophenotype is typically observed in germinal center-derived B-cell lymphomas?

    <p>CD19+, CD20+</p> Signup and view all the answers

    How does cyclophosphamide affect patients with diffuse large B-cell lymphoma?

    <p>It suppresses the immune response significantly.</p> Signup and view all the answers

    What is true about C-MYC translocation in the context of lymphomas?

    <p>It indicates a poor prognosis in B-cell lymphomas.</p> Signup and view all the answers

    Which of the following describes the morphology of cells in diffuse large B-cell lymphoma?

    <p>They tend to be large and pleomorphic.</p> Signup and view all the answers

    Which treatment is typically considered for relapse in diffuse large B-cell lymphoma?

    <p>Autologous stem cell transplant.</p> Signup and view all the answers

    What is a key feature of the ‘B symptoms’ noted in lymphoma patients?

    <p>Fever, night sweats, and unexplained weight loss.</p> Signup and view all the answers

    Study Notes

    Inherited Platelet Function Defects (Primary)

    • Impaired platelet adhesion due to endothelium failure to produce normal amounts of vWf (von Willebrand factor), Reduced factor VIII (vWf is carrier for VIII)
    • Mucosal bleeding (nosebleeds, dental work, heavy menses), ecchymoses, purpura, petechiae
    • Bernard-Soulier Syndrome: Impaired platelet adhesion, enlarged platelets, lack of platelet GPIb
    • Glanzmann Thrombasthenia: Impaired platelet aggregation, lack of platelet GPIIb/IIIa
    • Von Willebrand's Disease: Low vWF, low vWF activity, low factor VIII. Type 1 & 3 – deficient vWF; Type 2 – defective vWF. Autosomal dominant, chromosome 12
    • Treatment: Desmopressin (increases vWf release), recombinant vWF, cryoprecipitate

    Clotting Factor Deficiencies (Secondary Hemostasis)

    • Hemophilia A: Deficiency of factor VIII. X-linked recessive
    • Factor VII Deficiency, Factor X Deficiency, Factor V Deficiency, Factor II (prothrombin) Deficiency, Factor I (fibrinogen) Deficiency: Uncommon, mucosal or deep bleeds. Autosomal recessive inheritance pattern
    • Treatment: Factor VIII concentrates (plasma-derived or recombinant), cryoprecipitate

    Vitamin K Deficiency

    • Impaired platelet coagulation, prolonged PT (Prothrombin time) or PPT (partial thromboplastin time)
    • Can be caused by warfarin usage
    • Treatment: Hold warfarin and wait if stable, give vit K. If bleeding, administer factor concentrates, prothrombin complex concentrate, and plasma

    Disseminated Intravascular Coagulation

    • Platelet coagulation is impaired
    • Prolonged PT and PTT, decreased platelet count, decreased fibrinogen, increased D-dimers, increased fibrin split products
    • Underlying disorders can cause this
    • Treatment: Treat the underlying condition, supportive care

    Inherited Thrombophilia

    • Factor V Leiden: Most common thrombophilia, Factor V resistant to breakdown by activated protein C

    Thrombocytopenia Purpura

    • Life-threatening formation of blood clots in small vessels, microangiopathic hemolytic anemia
    • Symptoms: Fever, hemolytic anemia, renal dysfunction, thrombocytopenia, neuro dysfunction
    • Causes: ADAMTS13 absence/decrease (congenital or acquired), autoantibodies against phospholipid-binding proteins

    Acute Myeloid Leukemias (AML)

    • Most common adult leukemia, average age of diagnosis ~67
    • Symptoms: Fatigue, weakness, shortness of breath, fever/infections, bleeding/bruising, aches/pains, weight loss, night sweats
    • Diagnostic markers: Presence of myeloid lineage cells (CD33, CD13, MPO), at least 20% blasts/promyelocytes/promonocytes in bone marrow/blood
    • Treatment: 7+3 induction chemotherapy +/- stem cell transplant (high cure rates), hypomethylating agents for older patients

    Myelodysplastic Syndrome (MDS)

    • Clonal populations with defective maturation (cytopenias)
    • Often develops from cancer, radiation, exposure to benzene
    • High risk of progression to Acute Myeloid Leukemia (AML)
    • Treatment: Varies based on risk assessment; hypomethylating agents used in high risk, prevention of disease progression

    Chronic Myeloid Leukemia (CML)

    • Average age of diagnosis is ~60
    • Often asymptomatic, found through routine labs
    • Some symptoms: fatigue, malaise, splenomegaly (extramedullary hematopoiesis –abdominal pain, early satiety, weight loss)
    • Diagnostic marker: markedly increased WBC count, increased myeloid cells (especially neutrophils), basophilia, usually < 10% blasts
    • Treatment: Imatinib (TK inhibitor, tyrosine kinase inhibitor)

    Polycythemia Vera (PV)

    • Symptoms: Pruritus (itchiness), splenomegaly, hepatomegaly, erythromelalgia, arterial or venous thrombosis (common cause of death due to acute coronary syndrome)
    • Diagnostic marker: Elevated hemoglobin/hematocrit, decreased EPO
    • Treatment: Aspirin, phlebotomy, and hydroxyurea

    Essential Thrombocythemia (ET)

    • Symptoms: Elevated platelets, may progress to a spent phase (marrow fibrosis, splenomegaly) or AML.
    • Diagnostic marker: Elevated platelets, normo/mildly hypercellular marrow, lack of true fibrosis.
    • Treatment: Aspirin and observation (low risk); aspirin and hydroxyurea (high risk).

    Myelofibrosis

    • Replacement of marrow space by fibrosis without preceding myeloproliferative neoplasm (MPD)
    • Characterized by immature forms of cells produced into blood and worsening of anemia/thrombocytopenia.

    B-cell Acute Lymphoblastic Leukemia (ALL)

    • Usually leukemic, lymphoblasts fill the marrow
    • Symptoms: fever, bone pain, weakness, fatigue
    • Diagnosis: TdT and CD34 lymphoid markers. B-ALL diagnosed with CD10 and CD19

    T-cell Acute Lymphoblastic Leukemia (T-ALL)

    • Usually lymphomatous, mediastinal mass
    • Symptoms: fever, bone pain, weakness, fatigue
    • Diagnosis: TdT and CD34 Lymphoid markers, CD5, CD7, and CD3

    Mature Cell Lymphomas

    • Originates from germinal center and marginal zone B cells, bimodal age distribution (15–35 and >55), slightly more males

    Hodgkin Lymphoma

    • Continuous, orderly spread of disease (lymph node to spleen to liver to bone marrow)
    • Diagnosis: Reed-Sternberg cells (large, prominent nuclei and nucleolus, lots of cytoplasm) and reactive infiltrate
    • Subtypes: Nodular sclerosis (most common, low EBV association), Mixed cellularity (abundance cell mix, moderate EBV association), Lymphocyte rich (best prognosis, low EBV association), Lymphocyte depleted (worst prognosis, high EBV association)

    B-cell Lymphomas (High Grade/Aggressive)

    • Diffuse Large B-cell Lymphoma (DLBCL): Rapidly growing, doubling time under 24hrs, high incidence of tumor lysis syndrome
    • Burkitt Lymphoma: Rapidly fatal, increased incidence in immunocompromised individuals
    • Treatment: Varies greatly

    B-cell Lymphomas (Low Grade/Indolent)

    • Chronic Lymphocytic Leukemia/Small Lymphocytic Leukemia (CLL/SLL)
    • Mantle Cell Lymphoma (MCL): Aggressive disease presents in older adults (63 YO)
    • Follicular Lymphoma: Presents in older adults, involves lymph nodes, spleen, liver, bone marrow
    • Marginal Zone Lymphoma (MZL)

    Hairy Cell Leukemia

    • Originates in memory B cells
    • Common in elderly males
    • Often involves spleen, marrow, and blood
    • Diagnosis: TRAP stain, Hairy looking cells
    • Treatment: Cladribine (adenosine analog)

    Plasma Cell Neoplasms

    • Plasmacytoma: Bony lesions, can be found anywhere in the body
    • Multiple Myeloma: Infiltration of plasma cells into bone or other organs, anemia, bone pain, acute renal dysfunction, fatigue, hypercalcemia
    • Treatment: Radiation of localized tumor, chemo, stem cell transplant

    Immunoglobulin Abnormalities

    • Monoclonal Gammopathy of Uncertain Significance (MGUS): Asymptomatic, preneoplastic condition
    • Smoldering Myeloma: Asymptomatic, preneoplastic condition; elevated monoclonal protein

    Viral Associations

    • Adult T-cell Leukemia/Lymphoma (ATLL): HTLV-1 associated (endemic)
    • Extranodal NK/T-cell lymphoma

    Primary Amyloidosis

    • Impairment of kidney function, various hematological, neurological, and musculoskeletal issues
    • Diagnosis: Congo red stain

    Other

    • Langerhans Cell Histiocytosis (LCH)
    • Mycosis Fungoides/Sézary Syndrome(MF/SS)
    • Peripheral T-cell lymphoma (PTCL)

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    Description

    Test your knowledge on hematological disorders with this focused quiz on Myelofibrosis and B cell Acute Lymphoblastic Leukemia (B-ALL). Explore key mutations, cellular presentations, and treatment options. Perfect for medical students and professionals sharpening their expertise in hematology.

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