HEMATOLOGY Flashcards
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INT - 13.1
Please select from the list which contains the four classic myeloproliferative neoplams.
A) Hodgkin disease, chronic myeloid leukaemia, polycythaemia vera, hairy cell leukaemia
B) myelofibrosis, chronic myeloid leukaemia, polycythaemia vera, thrombocythaemia
C) thrombocythaemia, chronic myeloid leukaemia, polycythaemia vera, hairy cell leukaemia
D) polycythaemia vera, hairy cell leukaemia, agranulocytosis, myelofibrosis
ANSWER
B) myelofibrosis, chronic myeloid leukaemia, polycythaemia vera, thrombocythaemia
EXPLANATION
Chronic myeloproliferative disorders by defintion are different disorders of different myeloid cell lines, not including lymphoid system, so B is correct
INT - 13.2
The most common hereditary thrombophilia:
A) antithrombin deficiency
B) protein C deficiency
C) activated protein C resistence/Leiden-mutation
D) protein S deficiency
ANSWER
C) activated protein C resistence/Leiden-mutation
EXPLANATION
Leiden mutation is the most common genetic thrombophilia, so C is the correct answer.
INT - 13.3
You take care for a patient with deep vein thrombosis, which occured also previously too. Baseline aPTT prolonged with 12 seconds, which can not be corrected with addition of normal plasma. The most likely diagnosis:
A) dysfibrinogenaemia
B) lupus-anticoagulant/antiphospholipid syndrome
C) F XIII deficiency
D) antithrombin deficiency
ANSWER
B) lupus-anticoagulant/antiphospholipid syndrome
EXPLANATION
Dysfibrinogenaemia rarely cause thrombosis, if so mostly venous. Antithrombin deficiency does not typically provoke stroke, orherwise normal pasma can correct coagulation test results. In antiphospholipid lupus anticoagulant syndrome one might not achieve aPTT correction by mixing patient plasma with normal.
INT - 13.4
Which form of acute myeloid leukemia is most likely, if there is a severe general easy bruising, signs of acute DIC, Marrow smear contains Auer rods in myeloid precursory cells, and cytogenetics reveal translocation 15/17?
A) M1 undifferentiated myeloblastic
B) M2 differentiated myeloblastic
C) M3 promyelocytic
D) M4 myelomonocytic
E) M5 monocytic
ANSWER
C) M3 promyelocytic
EXPLANATION
Strong granulation of promyelocytes along with occasional Auer rods, acute DIC, and t15:17 translocation are hallmark findings of M3, acute promyelocytic leukemia
INT - 13.6
Only one of the lists fits to so called classical hemolytic uremic syndrome (HUS):
A) school aged kid, fever following couple of day a recent diarrhoea, moderate jaundice, mild renal failure, leukocytosis, thrombocytopenia, neurological signs and cognitive dysfunction
B) elderly lady, hypofibrinogenaemia, splenomegaly, acut DIC, thrombocytopenia, direkt bilirubin accumulation, oligo-anuria, apathy
C) gastric cancer patient, hyperfibrinogenaemia. low LDH activity, positive direct Coombs
ANSWER
A) school aged kid, fever following couple of day a recent diarrhoea, moderate jaundice, mild renal failure, leukocytosis, thrombocytopenia, neurological signs and cognitive dysfunction
EXPLANATION
Point A comprises standard clinical signs of classic hemolytic uremic syndrome . Splenomegaly, reduced fibrinogen level, elevated direct bilirubin are absent in HUS. Point C elements are atypical or never seen in HUS
INT - 13.7
Which alteration associates frequently with a moderate to severe form of congenital spherocytosis?
A) arteriosclerosis obliterans
B) immunodeficiency
C) gallstone disease
D) hypertension
E) diarrhoea
F) constipation
ANSWER
C) gallstone disease
EXPLANATION
Correct answer is C, bilirubin gallstone disease. No other organic consequences might be expected.
INT - 13.8
Which means Hodgin III/A Ann Arbor stage?
A) pathological lymph nodes both sides of the neck, axillary without fever and weight loss
B) pathological lymph nodes at the neck and inguinal region with fever and weight loss
C) pathological lymph nodes at the neck and inguinal region without fever and weight loss
D) pathological lymph nodes at the neck and supraclavicular region with fever and weight loss
ANSWER
C) pathological lymph nodes at the neck and inguinal region without fever and weight loss
EXPLANATION
Ann Arbor stage III/A is described under C.
INT - 13.9
Please identify the first line chemotherapy protocol of Hodgkin’s lymphoma.
A) CVP (cyclophosphamid + vincristin + prednisolon)
B) CAF (cyclophosphamid + adriablastin + fluorouracil)
C) TAD/HAM (thioguanin + Ara-C + daunorubicin/high dose Ara-C + mitoxantron)
D) ABVD (adriamycin + bleomycin + vinblastin + dacarbazin)
ANSWER
D) ABVD (adriamycin + bleomycin + vinblastin + dacarbazin)
EXPLANATION
ABVD is widely accepted as Hodgkin first line therapy. CAF and FUFA are used in breast and colon cancer, while TAD/HAM is an acute myeloid leukemia induction modality. Right answer is D.
INT - 13.10
Please identify a non-Hodgkin’s lymphoma in which lymphadenopathy und splenomegaly are not characteristic findings.
A) hairy cell leukemia
B) Waldenström-macroglobulinaemia
C) Burkitt-lymphoma
D) multiple myeloma
E) mantle-cell lymphoma
ANSWER
D) multiple myeloma
EXPLANATION
Correct is D, multiple myeloma, in which lymphadenopathy or splenomegaly occurs in less than 10%, myeloma cells are mainly occupying bone marrow. All the other listed entities are characterised by diffent degree of enlarged lymph nodes or spleen.
INT - 13.11
The most probable background of hypochromic microcyctic anemia:
A) pernicious anemia
B) anemia sideropenica
C) hyperthyreodism
D) agranulocytosis
ANSWER
B) anemia sideropenica
EXPLANATION
Good selection B. Anemia is mostly absent in hyperthyroidism and agranulocytosis. Megaloblastic morphology is typical to vitamin B12 deficiency
INT - 13.12
Hypersplenic hemopoesis can be characterised as:
A) leukocytosis with left shift splenomegaly
B) pancytopenia, portal hypertension, normal or increased reticulocyte count
C) thrombocytosis, splenomegaly
D) erythrocytosis, thrombocytosis, leukocytosis, splenomegaly
ANSWER
B) pancytopenia, portal hypertension, normal or increased reticulocyte count
EXPLANATION
B describes adequately hypersplenic hemopoiesis, in which pancytopenia usually develops along with portal hypertension, enlarged spleen and increased reticulocyte counts
INT - 13.13
Which hermolytic disorder is associated with pancytopenia?
A) spherocytosis
B) immunhemolytic anemia
C) sickle cell trait
D) paroxysmal nocturnal hemoglobinuria
E) thalassaemia minor
ANSWER
D) paroxysmal nocturnal hemoglobinuria
EXPLANATION
D is correct, the only hemolytic condition is paroxysmal nocturnal hemoglobinuria, which is a clonal hemopetic disorder affecting all cell lines.
INT - 13.14
Non pegylated factor concentrate half life in Hemophilia A
A) 2 hours
B) 6 hours
C) 12 hours
D) 24 hours
E) 48 hours
ANSWER
C) 12 hours
EXPLANATION
Factor VIII biological half life is 12 hours, so C is the right answer
INT - 13.15
Mild von Willebrand case with moderate bleeding or prevention only one of the agents efficient
A) pentoxyphyllin
B) calcium dobesilat
C) DDAVP- (desamino-D-arginin-vasopressin-) infusion
D) somatostatin
ANSWER
C)DDAVP- (desamino-D-arginin-vasopressin-) infusion
EXPLANATION
C is the most appropriate selection. Pentoxyphyllin and calcium dobesilate are not hemostatic agents. Somatostatin is an islet cell hormone possessing exclusively metabolic regulatory activities
INT - 13.16
In chilhood acute ITP prognosis:
A) If thrombocytopenia severe, spontaneous improvement is uncommon.
B) Spontaneous recovery is the most common.
C) Improvement might be achieved only with 1 mg/kg prednisone.
ANSWER
B) Spontaneous recovery is the most common.
EXPLANATION
The correct selection is B, as childhood acute ITP spontaneously and quickly cures in vast majoritiy of cases, without any intervention.
INT - 13.17
In chronic ITP (non post infectious event) the prognosis
A) Spontaneous remissions are the rule
B) Spontaneous remissions are uncommon, but after a short course of 1 mg/kg Prednisolon 80% achieves durable remissions
C) In chronic ITP spontaneous remissions are uncommon, first line 1 mg/kg Prednisolon achieves durable remissions in 10-15%
D) Any kind of remisssion needs combined immusuppressive therapy
ANSWER
C) In chronic ITP spontaneous remissions are uncommon, first line 1 mg/kg Prednisolon achieves durable remissions in 10-15%
EXPLANATION
C is correct, as in adultory ITP sponatenous cure is extremely rare. Point B is incorrect, as steroid therapy much less rarely results in longstanding remission in chronic ITP. Selection D is clearly incorrect, you do not neeed combined immunsuppression in ITP, as steroids, high dose invenous immunoglobulins, or is some cases Anti D sera might achieve remission, even if short duration.
INT - 13.18
Please identify the antidote of unfractionated (Na) heparin.
A) Vitamin K 20 mg iv.
B) Dicynon 20 mg iv.
C) ε-aminocapronic acid 4–8 g iv.
D) protamine sulphate
E) Styptanon
F) DDAVP
ANSWER
D) protamine sulphate
EXPLANATION
Good option is protamine sulphate, D, which is able to neutralise heparin. Vitamin K certainly not, it may be used in mild coumarin overdose. DDAVP can be efficient in mild von Willebrand disease
INT - 13.19
Megaloblastic (pernicious) anemia characteristics
1) Weak gastric acid output, only after pentagstrin stimulation.
2) Pentagastrin refractory achlorhydria in manifest anemia cases.
3) Normoblast accumulation in bone marrow.
4) Presence of giant metamyelocytes in the marrow and blood.
A) 1st, 2nd and 3rd answers are correct
B) 1st and 3rd answers are correct
C) 2nd and 4th answers are correct
D) only 4th answer is correct
E) all of the answers are correct
ANSWER
C) 2nd and 4th answers are correct
EXPLANATION
In megaloblastic (pernicious) anemia refractory achlorhydria is the rule. Nucleated erythroid precursors are large, so called megaloblastic cells, not normal sized normoblasts. So answer 1 and 3 are wrong. Giant metamyelocytes are frequently seen.
INT - 13.20
True feature(s) of megaloblastic anemia:
1) mild hemolysis with moderately increased indirect bilirubin
2) leukocytosis and trombocytosis
3) mild leukopenia and thrombocytopenia
4) normal LDH activity and hypocellular bone marrow
A) 1st, 2nd and 3rd answers are correct
B) 1st and 3rd answers are correct
C) 2nd and 4th answers are correct
D) only 4th answer is correct
E) all of the answers are correct
ANSWER
B) 1st and 3rd answers are correct
EXPLANATION
Disturbed DNA metabolism might induce leukopenia. Megaloblasts are in abundant amount in bone marrow, due to slow maturation their accumulation can be observed. This type of neuropathy may even be worse after folic acid- So the correct answers are 1 and 3.
INT - 13.21
The definition of severe hemophilia:
1) FVIII activity is 5–10%.
2) FVIII activity is less than 1%.
3) Petechiae and Rumpell-Leede test positivity are frequently observed.
4) Spontaneous haemarthros, joint bleedings are developing.
A) 1st, 2nd and 3rd answers are correct
B) 1st and 3rd answers are correct
C) 2nd and 4th answers are correct
D) only 4th answer is correct
E) all of the answers are correct
ANSWER
C) 2nd and 4th answers are correct
EXPLANATION
Severe hemophilia by defintion means Factor VIII activity less than 1% of normal. Petechiae are present in low platelet count or vasculitis conditions. So Answer 2 and 4 are the good ones.
INT - 13.22
Identify the correct response combinations in hemophilia A.
1) Plasma von Willebrand is normal.
2) Plasma von Willebrand-factor is decreased.
3) Prolonged aPTT which can be corroected with normal plasma mixing.
4) aPTT is prolonged, but thrombin time is even more prolonged.
A) 1st, 2nd and 3rd answers are correct
B) 1st and 3rd answers are correct
C) 2nd and 4th answers are correct
D) only 4th answer is correct
E) all of the answers are correct
ANSWER
B) 1st and 3rd answers are correct
EXPLANATION
Decreased Wiilebrand factor induce von Willebrand disease and not hemophilia. Thrombin clotting time is not altered by hemophilia A, since thist test measures fibrinogen polymerisation induce by thrombin, so it is not affected hemophilic condition. aPTT can corrected by addition of normal plasma in hemophilia, so the right selection are 1 and 3.
INT - 13.23
True features of agranulocytosis (febrile neutopenia):
1) mostly drug associated
2) mostly idiopathic
3) no erythropetic or thrombopoietic abnormalities are present, myeloid maturation is blocked at early stage
4) there is a pancytopenia and severe bone marrow hypocellularity
A) 1st, 2nd and 3rd answers are correct
B) 1st and 3rd answers are correct
C) 2nd and 4th answers are correct
D) only 4th answer is correct
E) all of the answers are correct
ANSWER
B) 1st and 3rd answers are correct
EXPLANATION
Agranulocytosis or (febrile neutropenia) almost exclusively induced certain drugs, and appears isolated. So the correct answers are 1 and 3
INT - 13.25
Characteristics of FVII deficiency:
1) rare condition, not inducing bleeding events
2) rare condition with severe bleedings
3) prolonged aPTT, thrombin time, along with prothrombin time, which all can be corrected by addition of normal plasma
4) Isolated prothrombin time prolongation which can not be corrected by iv, vitamin K
A) 1st, 2nd and 3rd answers are correct
B) 1st and 3rd answers are correct
C) 2nd and 4th answers are correct
D) only 4th answer is correct
E) all of the answers are correct
ANSWER
C) 2nd and 4th answers are correct
EXPLANATION
Facor VII deficiency carries high bleeding risk, including gastrointestinal tract as well. It is characterised by isolated spontaneous prothrombin time prolongation (Normal aPTT and TT), and this prolangation could not be corrected by iv vitamin K administrarion. Right answers are 2 and 4
INT - 13.26
Typical attribute(s) of chronic lymphocytic leukemia:
1) long disease course with good therapeutic responses, no refractory cases
2) durable course, rather indolent features, sometimes only partial responses to therapies, CD5/CD19 coexpressed cell count exceeds 5 g/L in peripheral blood
3) immunglobulins are elevated
4) low immunoglobulin levels
A) 1st, 2nd and 3rd answers are correct
B) 1st and 3rd answers are correct
C) 2nd and 4th answers are correct
D) only 4th answer is correct
E) all of the answers are correct
ANSWER
C) 2nd and 4th answers are correct
EXPLANATION
Chronic lymphoid leukemia (CLL) sometimes has less duration than other CLL cases. In al cases CD5/C19 coexpression cells are present, the response therapy might be partial. However acquired immunoglobulin, especially IgG deficiency is usually present sooner or later in all cases. Good selections are answer 2 and 4.
INT - 13.27
What is/are diagnostic criterium of chronic lymphocytic leukemia?
1) Fever, painful lymphadenopathy, elevated CRP
2) Leukocytosis with left shift
3) Monoclonal gammopathy
4) CD5/CD19 coexpression cell count exceeds 5 G/L in peripheral blood
A) 1st, 2nd and 3rd answers are correct
B) 1st and 3rd answers are correct
C) 2nd and 4th answers are correct
D) only 4th answer is correct
E) all of the answers are correct
ANSWER
D) only 4th answer is correct
EXPLANATION
How chronic lymhphocytic leukemia is diagnosed, what are the criteria? The only really diagnostic criterium is in point D, CD19/CD5 coexpressed 5 G/L cell count in peripheral blood. In chronic lymphoid leukemia left shit of white blood cell, painful or inflammed lymph nodes, monoclonal protein accumulation are absent.
INT - 13.28
Therapies which are able to prolong multiple myeloma survival:
1) per os melphalan plus prednisolon
2) bortezomib, proteasome inhibitors
3) vincristine
4) dexamethasone
5) high dose melphalan with autologous stem cell transplantation
6) thalidomide, revlimide
7) antracyclines
8) methotrexate
A) 2nd, 5th and 6th answers are correct
B) 3rd, 6th and 8th answers are correct
C) 2nd, 4th and 7th answers are correct
ANSWER
A) 2nd, 5th and 6th answers are correct
EXPLANATION
Only autologous transplatation, proteasome inhibitors (bortezomib) and imids are able prolong substantialy survival in myeloam, all the other agents listed are not. So A is the good response.
INT - 13.29
ITP attributes:
1) isolated thrombocytopenia
2) pancytopenia and thrombocytopenia
3) significant splenomegaly
4) splenomegaly is usually absent, however, steroid refractory cases may respond with 60-70% durable remission rate to splenectomy
5) template bleeeding time prolonged
6) prolonged clotting times
A) 1st, 4th and 5th answers are correct
B) 2nd and 3rd answers are correct
C) 2nd, 3rd and 6th answers are correct
ANSWER
A) 1st, 4th and 5th answers are correct
EXPLANATION
In ITP thrombocytopenia is isolated without other blood count abnormalities. Spleen is not really substantially enlarged, steroid refractory cases may respont to splenectomy in 60-70%. Bleeding time is prolonged
INT - 13.30
Heparin complications
1) Heparin induced thrombocytopenia (HIT) is a mild complication, may inducse some easy bruising, injection site reactions
2) HIT is frequently sever complication, runs high mortality, serious bleeding and simultaneous progressive thrombosis may develop
3) HIT is more common with low molecular weight heparins
4) HIT is more common with unfractionated heparin dervatives.
5) Heparin may induce cholestatic enzym elevations
6) Heparin may induce transaminase elevation.
A) 1st., 2nd and 3rd answers are correct
B) 1st., 3rd and 5th answers are correct
C) 2nd., 4th and 6th answers are correct
ANSWER
C) 2nd., 4th and 6th answers are correct
EXPLANATION
Heparin induced thrombocytopenia runs high mortality with simultaneous venous aretrial thrombotic events and major bleedings. It occurs more frequently with unfractionated heparin. transaminase might be elevated during heparin therapy.
INT - 13.31
Poor prognostic moments in Hodgkin lymphoma:
1) Lymphoid depletion type
2) Lymphoid predominant
3) Ann Arbor IV stage
4) Ann Arbor I stage
5) Age over 45
6) Age under 45
7) Interim PET CT shows progression
8) Stagnation or moderate improvement on interim PET CT
A) 1st, 3rd, 5th and 7th answers are correct
B) 2nd and 4th answers are correct
C) 6th and 8th answers are correct
D) 2nd, 4th, 6th and 8th answers are correct
ANSWER
A) 1st, 3rd, 5th and 7th answers are correct
EXPLANATION
Lymhoid depletion and male sex, age over 45 years comes with worse prognosis in Hodgkin. Interim PETCT after 3-4 cycles of chemotherapy may show progression, which clearly indicates poor prognosis, refractory disease
INT - 13.33
Please select agents convenient to treat acute or subacute arterial thrombosis.
1) Coumarol
2) Pentoxyphyllin
3) Aspirin
4) Clopidogrel
5) tissue plasminogen activator
6) low molecular weight heparin
7) ε-aminokapron acid
8)DDAVP(desamino-D-arginin-vasopressin)
A) 1st and 2nd answers are correct
B) 3rd, 4th, 5th and 6th answers are correct
C) 7th and 8th answers are correct
D) all of the answers are correct
ANSWER
B) 3rd, 4th, 5th and 6th answers are correct
EXPLANATION
Coumarols are effective in venous thrombotic event prevention, not in arterial. Pentoxyphyllin might improve microcirculation only. Epsilon amino capronic acid is blocking fibrinolysis. So agents in 3,4,5,6 are the correct selection
INT - 13.35
Polycythemia vera typical characteristics
1) leukocytosis, thrombocytosis Jak V617F mutation negative, splenomegaly
2) leukocytosis, thrombocytosis, moderate splenomegaly JAK-V613F mutation
3) normal leukocyte and platelet counts, oxigen saturation under 90%
4) after a longer period myelofibrotic transformation may occurr
5) Needs no intervention in early stage, phlebotomy is indicated, if elevated platelets counts are persistent
6) Early combined antacyclin+cytosin arabinoside based combined induction therapy seems to the most efficacious, so this could be selected
A) 1st and 5th answers are correct
B) 3rd and 6th answers are correct
C) 2nd and 4th answers are correct
ANSWER
C) 2nd and 4th answers are correct
EXPLANATION
Leukocytosis and elevated platelet counts are typical in polycythemia, spleen is usually moderately enlarged, and JAK2 type mutation is present in 95% of patients. After 12-15 years myelofibrotic transformation may develop. Early stage with elevated hematocrit certainly needs phlebotomy or other interventions to avoid vascular complications. Correct selection 2 and 4.
INT - 13.36
Identify typical coumarol complications:
1) thrombocytopenia
2) skin or intestinal necrosis
3) diarrhoea, abdominal distension
4) agranulocytosis
5) if INR over 5, spontaneous bleedings
6) spontaneous bleedings with INR over 2
A) 1st, 3rd and 4th answers are correct
B) 2nd and 5th answers are correct
C) 4th and 6th answers are correct
ANSWER
B) 2nd and 5th answers are correct
EXPLANATION
Coumarols are not provoking thrombocytopenia or leukopenia. Diarrhoea and abdominal distension are also absent. Intestinal necrosis might occur, and if INR is over 5,0 severe bleeding complications may develop.
INT - 13.37
Unfractionated heparin monitoring feasible tools
1) prothrombin time
2) aPTT
3) global clotting time measured with appropriate bedside device
4) urea lysability test
5) APC- resistance
A) 1st and 4th answers are correct
B) 1st and 5th answers are correct
C) 2nd and 3th answers are correct
D) 4th and 5th answers are correct
ANSWER
C) 2nd and 3th answers are correct
EXPLANATION
Unfractionated heparin prolongs both aPTT and thrombin time, and also global clotting time. APC resistance is a method to screen thrombophilia, urea clot lysability test is an old screening approach to screen for FXIII deficiency.
INT - 13.38
Please select condition predisposing to arterial thrombosis
1) FV. Leiden mutation
2) hyperhomocysteinaemia
3)lupus-anticoagulant/antiphospholipid-antibody syndrome
4) antithrombin deficiency
5) unstable angina
6) Prinzmetal angina
7) long lasting thrombocytosis
8) reduced blood viscosity
9) immobilisation
A) 1st, 4th and 6th answers are correct
B) 2nd, 3rd, 5th and 7th answers are correct
C) 4th, 6th, 8th and 9th answers are correct
ANSWER
B) 2nd, 3rd, 5th and 7th answers are correct
EXPLANATION
Leiden mutation, antithrombin or protein C deficiency, immobilisation predispose to venous thrombotic events. Reduced viscosity protects from thrombosis. 2,3,5,7 are the right selections.
