Quiz bee Flashcards
Cytogenetic clonal evolution in CML is usually associated with which chromosomal abnormalities?
inv(16), del11q, trisomy 8, monosomy 7
These abnormalities indicate the progression of Chronic Myeloid Leukemia (CML).
Which TKI is associated with higher rates of hyperglycemia, pruritus, skin rashes and hyperbilirubinemia?
Nilotinib
Nilotinib is a second-generation tyrosine kinase inhibitor (TKI) used in CML treatment.
What is a criterion of accelerated phase in CML?
> 5% peripheral blast
Other criteria include WBC count of > 500 x 10^9/L and > 20% peripheral basophils.
Which surface or cytoplasmic markers are seen in acute lymphoblastic leukemia of T cell lineage?
CD3
CD3 is a pan-T cell marker that indicates T cell lineage.
Which subclass of ALL does not require maintenance chemotherapy?
T-ALL
T-ALL is a type of acute lymphoblastic leukemia that generally has a different treatment protocol.
Which cytogenetic abnormality is associated with the poorest prognosis in CLL?
del (17)
Deletion of chromosome 17 is linked to aggressive disease behavior in chronic lymphocytic leukemia (CLL).
What is the immunophenotype most consistent with CLL?
CD5 (+), CD10 (-), CD19 (+), CD20 (+), CD 23 (+)
This specific marker profile is used to identify CLL in laboratory diagnostics.
Which parameter is not included in the Binet Staging System for CLL?
Platelet count
The Binet staging system considers lymph node involvement, hemoglobin levels, and WBC count.
Which lymphoma is known to harbor t(2;5)?
Anaplastic Large Cell Lymphoma
This translocation is characteristic of anaplastic large cell lymphoma.
Which lymphoma is most commonly associated with a monoclonal IgM paraprotein?
Lymphoplasmacytic Lymphoma
This type of lymphoma is often linked with Waldenström’s macroglobulinemia.
Which agent can affect stem cell collection and should be avoided in initial therapy for transplant-eligible myeloma patients?
Daratumumab
Daratumumab can interfere with stem cell mobilization.
Which combination agent is used as maintenance therapy for myeloma patients with high-risk cytogenetics?
Lenalidomide and Cyclophosphamide
This combination is effective for maintaining remission in high-risk patients.
Daratumumab specifically targets which surface marker?
CD38
CD38 is a marker commonly expressed on myeloma cells.
Which is not a known precursor protein for Familial Amyloidosis?
Fibrinogen Aα
Known precursor proteins include Gelsolin and Transthyretin.
Which subtype of light chains in Amyloidosis are predisposed to fibril formation in the kidneys?
Lambda 6
Specific light chain subtypes have different risks for amyloid fibril formation.
Macroglossia, a pathognomonic sign in Light Chain Amyloidosis is seen in what percentage of patients?
10%
Macroglossia is a notable clinical feature but occurs in a minority of cases.
Congestive Heart Failure due to Amyloid Cardiomyopathy is best treated by which agent?
Carvedilol
Carvedilol is a beta-blocker used to manage heart failure symptoms.
Cold Agglutinins secondary to Mycoplasma infection are directed to antigens of which blood group system?
I
This system is associated with cold agglutinin disease.
Acanthocytosis, hemolytic anemia, muscular dystrophy and cardiac defects is part of a phenotype seen in absence of which RBC antigen?
Kell
The Kell antigen is important for red blood cell integrity.
What is the most frequent reaction associated with blood transfusion?
Febrile Non-Hemolytic
This is the most common type of transfusion reaction.
Which is NOT a recipient risk factor for development of Transfusion Associated Lung Injury (TRALI)?
Chronic Alcohol Use
Risk factors include multiparity and smoking.
Clonal Eosinophilia exhibiting the FIP1L1-PDGFRA mutation is best treated by which agent?
Imatinib
Imatinib is effective for this specific mutation in eosinophilia.
Systemic mastocytosis is often associated with mutations involving which gene?
KIT
KIT mutations are common in systemic mastocytosis.
Which organ systems are not included in the clinical staging and grading of Acute Graft-Versus-Host Disease?
Respiratory
Clinical staging typically includes gastrointestinal, hepatobiliary, and integumentary systems.
Plerixafor is employed to augment mobilization of stem cells by antagonizing which receptor?
CXCR4
CXCR4 is a chemokine receptor crucial for stem cell homing.
Thrombocytopenia occurring within 24 hours of drug administration is characteristic of which medication?
Abciximab
Abciximab is a glycoprotein IIb/IIIa inhibitor.
Testing for anti-heparin/PF4 antibodies should be considered for individuals with suspected HIT and a minimum score of what in the 4T scoring system?
4
A score of 4 indicates high probability of heparin-induced thrombocytopenia (HIT).
Of all therapeutic modalities in ITP, which has the highest long-term response rate?
Splenectomy
Splenectomy is often curative for ITP.
Which disease of inherited thrombocytopenia is inherited through an X-linked pattern?
Wiskott-Aldrich Syndrome
This syndrome is characterized by eczema, recurrent infections, and thrombocytopenia.
Medication related microangiopathic hemolytic anemia secondary to antibody formation is most commonly associated with which medication?
Ticlopidine
Ticlopidine is known to cause this type of hemolytic anemia.
Acquired platelet dysfunction due to uremia can be improved by increasing the hematocrit to a minimum of how much?
27%
Maintaining hematocrit above this threshold can alleviate symptoms.
Which subtype of von Willebrand disease is associated with a gain-of-function mutation leading to increased spontaneous binding of VWF to platelets?
2B
This subtype is characterized by enhanced platelet aggregation.
Heyde’s syndrome seen in individuals with an acquired form of VWD is associated with pathology of which cardiac valve?
Aortic
Heyde’s syndrome relates to aortic stenosis and angiodysplasia.
95% of all cases of Classical Hodgkin Lymphoma are of what histologic subtype?
Nodular Sclerosis and Lymphocyte Rich
This subtype is the most prevalent in Hodgkin lymphoma cases.
Which clinical parameter is not utilized in the IPS scoring system for advanced stage Hodgkin Lymphoma?
Lymphocyte count
The IPS includes albumin, ESR, and hemoglobin.
Which coagulation factor is a transglutaminase that cross-links and stabilizes fibrin clot?
Factor XIIIa
Factor XIII plays a critical role in blood coagulation.
What is the minimum dose of Vitamin K for individuals on chronic warfarin therapy with INR >10?
2 mg
This dose is recommended in the absence of significant bleeding.
Which clinical trial involving venous thromboembolism and direct oral anticoagulants is correctly matched?
RECOVER : Rivaroxaban
Each trial validates the efficacy of specific anticoagulants.
Deficiency of which clotting factor is most commonly associated with an isolated prolongation of the prothrombin time?
Factor VII
Factor VII deficiency leads to prolonged PT without affecting aPTT.
Which protein expressed by diffuse large B cell lymphoma (DLBCL) is associated with a germinal center origin?
CD10
CD10 is a marker indicating germinal center origin in DLBCL.
Indolent variants of Mantle Cell Lymphoma are known to express which protein?
SOX11
SOX11 expression is a characteristic feature of certain mantle cell lymphomas.
Which interleukin is considered the candidate molecule for the etiology of paraneoplastic thrombocytosis?
IL-6
IL-6 plays a role in the inflammatory response and thrombocytosis.
Which hematologic malignancy is responsive but cannot be cured by standard chemotherapy?
Chronic Lymphocytic Leukemia
CLL can be managed but often remains incurable.
Which vaccine is contraindicated for patients with hematologic malignancies undergoing intensive chemotherapy?
Measle/mumps/rubella
Live attenuated vaccines pose risks in immunocompromised patients.
What is the most striking coagulation test abnormality in severe COVID-19 patients?
Elevated d-dimer
Elevated d-dimer levels indicate increased thrombotic activity in COVID-19.
Which novel oral anticoagulants used in the treatment of VTE require an initial 5-day course of parenteral anticoagulation?
Dabigatran and Edoxaban
These require bridging therapy due to their mechanism of action.
A target joint in patients with hemophilia is defined by the occurrence of __ or more spontaneous bleeds in the same joint within a __ month period.
3;6
This definition is used to assess joint health in hemophilia.
Transfusion-Associated Graft-Versus-Host Disease can be prevented by which modification in transfused blood products?
Irradiation
Irradiation helps prevent GVHD by inactivating T lymphocytes.
Which chromosomal abnormalities seen in Multiple Myeloma is considered to be a high risk category?
t(4;14)
This translocation is associated with aggressive myeloma features.
Which abnormality is not typically seen in Thrombotic Thrombocytopenic Purpura?
Elevated haptoglobin
TTP typically presents with low haptoglobin levels.
Which cytogenetic abnormalities seen in Myelodysplastic Syndrome is most often associated with thrombocytosis?
del (5q)
This deletion correlates with increased platelet counts.
Which of the following abnormalities is not typically seen in Thrombotic Thrombocytopenic Purpura?
Elevated haptoglobin
Increased LDH, indirect hyperbilirubinemia, and polychromasia are typically seen in TTP.
Which of the following cytogenetic abnormalities seen in Myelodysplastic Syndrome is most often associated with thrombocytosis?
del (5q)
Other abnormalities listed may be present but are not typically associated with thrombocytosis.
DLBCL with extranodal involvement of which of the following organs is associated with an increased risk of CNS dissemination?
Testes
This association highlights the need for monitoring CNS involvement in patients with DLBCL.
A patient who is capable of only limited self-care and confined to the bed or chair >50% of waking hours is considered to have what ECOG score?
Grade 3
ECOG scores range from 0 (fully active) to 5 (dead), with Grade 3 indicating significant limitations.
Blinatumomab is a bispecific monoclonal antibody used in relapsed B-ALL that targets which of the following cell surface markers?
CD19 and CD3
This dual targeting helps redirect T cells to attack B-cell malignancies.
Which of the following ABO blood types is associated with a lower level of Von Willebrand Factor?
Type O
This has implications for bleeding disorders and management strategies.
Which of the following abnormalities seen in liver disease will favor a thrombotic phenotype?
Decreased ADAMTS13
ADAMTS13 is crucial for regulating von Willebrand factor and its deficiency can lead to thrombosis.
Which of the following abnormalities seen in liver disease will favor a bleeding phenotype?
Decreased antithrombin
Antithrombin is a key anticoagulant protein, and its decrease can lead to bleeding tendencies.
TRUE or FALSE: Urinary alkalinization with sodium bicarbonate is a recommended management in the prevention of tumor lysis syndrome by increasing uric acid solubility.
TRUE
This management strategy is utilized to mitigate the risk of tumor lysis syndrome.
TRUE or FALSE: Pulse Oximetry is the most accurate way of assessing oxygenation in patients with hyperleukocytosis.
FALSE
Pulse oximetry may be inaccurate in the setting of extreme leukocytosis.
TRUE or FALSE: The lumbosacral spine is the most common site of spinal cord compression due to metastatic tumors.
TRUE
Awareness of this common site is crucial for timely diagnosis and intervention.
TRUE or FALSE: The most significant chest radiograph finding in Superior Vena Cava Syndrome is widening of the superior mediastinum, most commonly on the right side.
TRUE
This finding is often indicative of obstruction in the superior vena cava.
TRUE or FALSE: Acute leukoencephalopathy seen in ALL patients receiving methotrexate is a dose-related toxicity characterized by somnolence and confusion that is often irreversible.
TRUE
This toxicity is a significant concern in the management of ALL.
Anthracycline cardiotoxicity occurs through a root mechanism of chemical free-radical damage mediated by which element?
Iron
Iron plays a critical role in the generation of free radicals that damage cardiac tissue.
Which of the following classes of chemotherapeutic agents is associated with the highest rates of male and female infertility?
Alkylating agents
Alkylating agents can cause significant gonadal toxicity.
Which of the following parameters of lung function is considered to be a sensitive measure of toxicity and recovery in patients with Bleomycin induced pneumonitis?
DLCO
Diffusing capacity of the lungs for carbon monoxide is a key indicator of lung health.
Identification: Symptoms of hyperviscosity in Multiple Myeloma occur at a level greater than _____ centipoise (cP).
4/four
This threshold is critical for recognizing hyperviscosity syndrome.
Identification: Congenital or Inherited deficiency of ADAMTS13 is also known as ________ syndrome.
Upshaw-Schulman
This syndrome is associated with Thrombotic Thrombocytopenic Purpura.
What is the most distinctive finding in patients who have Gamma Heavy Chain Disease (also known as Franklin’s Disease)?
Palatal Edema
This finding is characteristic of this rare plasma cell disorder.
Iron Refractory Iron Deficiency Anemia (or IRIDA) is characterized by a mutation in which of the following proteins that affects iron homeostasis?
Tmprss6
This protein is crucial for regulating hepcidin and iron absorption.
Which of the following recurrent genetic abnormalities in AML is correctly matched to its corresponding leukemogenic fusion genes?
t(9;11): MLLT3-KMT2A
This fusion is one of the significant genetic abnormalities in acute myeloid leukemia.
Which of the following CBC parameters has been reported as a risk factor for all-cause mortality in population-based studies?
RDW
Red cell distribution width is a valuable prognostic marker.
Howell-Jolly bodies are dense blue circular inclusions that represent which of the following?
Nuclear remnants
These bodies indicate a lack of splenic function or post-splenectomy status.
Spherocytes can be seen in the peripheral blood smear of patients infected with which of the following organisms?
Malaria spp.
Spherocytes are often associated with hemolytic anemias.
Which of the following RBC inclusions represent aggregated rough endoplasmic reticulum?
Basophilic stippling
This finding is often seen in conditions like lead poisoning.
The presence of a large number of RBCs with an area of central pallor that contain a dense center is typical of which of the following conditions?
Hemoglobin C disease
This morphological change is characteristic of this hemoglobinopathy.
Which of the following is incorrectly paired?
Polychromatophilia: ribosomal DNA
Polychromatophilia actually indicates the presence of ribosomal RNA.
How many percent of the circulating red cells is/are replaced daily by normal red cell production?
0.8-1%
This reflects the normal turnover rate of red blood cells.
Below what hemoglobin concentration does plasma EPO level increase in proportion to the severity of anemia?
6-9 g/dL
EPO response is critical in managing anemia.
Mean cell hemoglobin is calculated using which of the following formulas?
(Hemoglobin x 10)/Hematocrit
This calculation helps in assessing the average hemoglobin content in red cells.
Some iron in tissue stores is meant at which serum ferritin level?
50 ug/L
This level indicates adequate iron stores in the body.
The following disease conditions are associated with a low reticulocyte production index, except:
Hemoglobinopathy
Hemoglobinopathies typically do not result in a low reticulocyte index.
EPO deficiency may be more marked than would be predicted by the degree of renal insufficiency in which of the following disease conditions?
Aplastic anemia
In aplastic anemia, EPO levels may not correlate with renal function.
Nuclear maturation defects result from the following, except?
Pyridoxine deficiency
Pyridoxine deficiency is not typically associated with nuclear maturation defects.
Blood viscosity increases logarithmically at what hematocrit level?
40%
This threshold is important for understanding blood flow dynamics.
Severe chronic neutropenia is associated with mutations in which of the following genes?
ELANE
ELANE mutations are implicated in congenital neutropenia syndromes.
Which of the following is not characteristic of Felty’s syndrome?
Cytotoxic large granular lymphocytes
Felty’s syndrome typically involves splenomegaly and rheumatoid arthritis.
Patients with chronic granulomatous disease have increased infections secondary to which of the following organisms?
Burkholderia cepacia
This organism is one of the notable pathogens in these patients.
Eosinophilia-myalgia syndrome is a multisystem disease with prominent cutaneous, hematologic, and visceral manifestations. It is caused by ingestion of products containing which of the following?
L-tryptophan
This syndrome is linked to the consumption of contaminated L-tryptophan supplements.
What is the major protease enzyme of the fibrinolytic system?
Plasmin
Plasmin plays a crucial role in breaking down fibrin in blood clots.
Which of the following can inhibit protein kinase C-mediated platelet aggregation?
Omega-3 fatty acid
Omega-3 fatty acids have cardioprotective effects and can modulate platelet function.
The following conditions cause prolonged activated partial thromboplastin time (aPTT) and prothrombin time (PT), except:
Factor IX deficiency
Factor IX deficiency typically affects aPTT but not PT.
The following diseases are associated with massive splenomegaly, except:
Polycythemia vera
Massive splenomegaly is more characteristic of hematological malignancies.
How many days does it take for hematopoietic progenitors to become mature cells?
5-10 days
This timeframe is crucial in understanding hematopoiesis.
A 35-year-old nuclear bomb casualty was referred for pancytopenia. In the absence of extraordinary support measures, he is expected to survive in how many weeks?
2-4 weeks
This highlights the severity of pancytopenia post-radiation exposure.
Hematopoietic cells lose their proliferative capacity as they differentiate. Which of the following cells is an exemption to this rule?
Lymphoid cells
Lymphoid cells can continue to proliferate in response to antigenic stimulation.
Which of the following has the highest affinity for transferrin receptors?
Diferric transferrin
Diferric transferrin is the most effective form for iron transport in the body.
How many milligrams of elemental iron does a female in the child-bearing years need to absorb daily to meet her needs?
1.4 mg
This amount is essential for maintaining iron homeostasis during menstruation.
Which of the following is consistent with iron-deficient erythropoiesis?
serum ferritin <15 ug/L, serum iron <30, TSAT <10%, microcytic RBC morphology
These parameters indicate a clear state of iron deficiency.
Ring sideroblasts are caused by accumulation of iron in which cellular organelle?
Mitochondria
This accumulation is significant in certain types of anemia.
Aside from serum ferritin, what laboratory test has been proposed to distinguish between iron deficiency and anemia of inflammation?
Transferrin receptor protein
This test can help differentiate between the two conditions.
A dose of 200mg of elemental iron per day results in the absorption of how many mg iron?
25 mg/d
This absorption rate is typical for oral iron supplementation.
Which of the following toxins can cause acquired methemoglobinemia by oxidizing heme iron?
Nitrate
Nitrate exposure can lead to significant health concerns, including methemoglobinemia.
Which of the following drugs can cause megaloblastic anemia by lowering transcobalamin I?
Metformin
This effect is particularly relevant in the management of diabetic patients.
What is the recommended daily dose of folic acid in a pregnant woman with a previous fetus with neural tube defect?
400 ug
This recommendation is aimed at preventing neural tube defects in future pregnancies.
Which of the following etiologies of hemolytic anemia is due to an acquired intracorpuscular defect?
Paroxysmal nocturnal hemoglobinuria
This condition is characterized by hemolysis due to complement-mediated lysis.
Which of the following is the most abundant red cell membrane protein?
Spectrin
Spectrin provides structural support to the red blood cell membrane.
Which of the following genes is implicated in Southeast Asia Ovalocytosis (SAO)?
SLC4A1
Mutations in this gene are associated with the oval shape of red blood cells in this condition.
Which of the following is not a characteristic of cold agglutinin disease?
Antibodies produced by T lymphocyte clone
Cold agglutinin disease typically involves IgM antibodies produced by B cells.
Which of the following drugs is effective in reversing anemia in MDS patients with 5q- syndrome?
Lenalidomide
This drug is specifically indicated for patients with this genetic abnormality.
Chronic neutrophilic leukemia has been associated with what chromosomal translocation?
t(9;22)
This translocation is significant in the pathogenesis of this leukemia.
Which of the following myeloproliferative neoplasms express JAK 2 mutation in more than 95% of cases?
Polycythemia vera
This mutation is a hallmark of polycythemia vera.
At what chromosome number is the JAK2 gene located?
Chromosome 9
This gene is crucial for hematopoietic signaling.
Which of the following drugs used to reduce platelet count lacks marrow toxicity and has protective effects against venous thrombosis?
Anagrelide
Anagrelide is specifically used in the management of essential thrombocythemia.
Transformation to an aggressive form of acute leukemia occurs spontaneously in how many percent of patients with PMF?
20%
This transformation poses significant challenges in the management of primary myelofibrosis.
Which chromosome is associated with Down syndrome?
Chromosome 21
Down syndrome is caused by an extra copy of chromosome 21, known as trisomy 21.
Which drug used to reduce platelet count lacks marrow toxicity?
Anagrelide
Anagrelide is known for its protective effects against venous thrombosis.
What percentage of patients with PMF spontaneously transform to an aggressive form of acute leukemia?
20%
This transformation occurs in a notable subset of patients with primary myelofibrosis (PMF).
Down-syndrome associated AML in young children is typically of what subtype?
Acute megakaryocytic leukemia
This subtype is particularly linked to children with Down syndrome.
Which of the following is NOT an AML-defining recurrent genetic abnormality?
del(5q)
The other listed abnormalities are well-established markers for acute myeloid leukemia.
Myeloid sarcomas are associated with which chromosomal abnormality?
t(15;17)
This translocation is commonly associated with acute promyelocytic leukemia as well.
Which genetic abnormality is associated with abnormal bone marrow eosinophils?
t(15;17)
This abnormality is linked to acute promyelocytic leukemia, which can affect eosinophil levels.
Cytarabine is specific to which phase of the cell cycle?
S phase
Cytarabine interferes with DNA synthesis primarily during the S phase.
Which of the following is NOT a FLT3 inhibitor?
Vorinostat
Vorinostat is a histone deacetylase inhibitor, not a FLT3 inhibitor.
Which of the following is NOT a CD20 targeting antibody?
Alemtuzumab
Alemtuzumab targets CD52, not CD20.
What is the only adult leukemia not related to radiation?
CML
Chronic myeloid leukemia (CML) is primarily associated with the Philadelphia chromosome.
Which blood type is associated with increased incidence of COVID-19 infection?
Type A blood
Studies have suggested that individuals with type A blood may have a higher risk of severe COVID-19.
