Internal Medicine - Hematology

Question 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 1

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

Question 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 2

C) activated protein C resistence/Leiden-mutation

EXPLANATION
Leiden mutation is the most common genetic thrombophilia, so C is the correct answer.

Question 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 3

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.

Question 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 4

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

Question 5

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 5

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

Question 6

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 6

C) gallstone disease

EXPLANATION
Correct answer is C, bilirubin gallstone disease. No other organic consequences might be expected.

Question 7

Which means Hodgin III/A Ann Arbor stage?

A) pathological lymph nodes on 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 7

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.

Question 8

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 8

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.

Question 9

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 9

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.

Question 10

The most probable background of hypochromic microcyctic anemia:

A) pernicious anemia
B) anemia sideropenica
C) hyperthyreodism
D) agranulocytosis

Answer 10

B) anemia sideropenica

EXPLANATION
Good selection B. Anemia is mostly absent in hyperthyroidism and agranulocytosis. Megaloblastic morphology is typical to vitamin B12 deficiency

Question 11

Hypersplenic hemopoiesis can be characterized 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 11

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

Question 12

Which hemolytic disorder is associated with pancytopenia?

A) spherocytosis
B) immunhemolytic anemia
C) sickle cell trait
D) paroxysmal nocturnal hemoglobinuria
E) thalassaemia minor

Answer 12

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.

Question 13

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 13

C) 12 hours

EXPLANATION
Factor VIII biological half life is 12 hours, so C is the right answer

Question 14

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 14

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

Question 15

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 15

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.

Question 16

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 remission needs combined immunsuppressive therapy

Answer 16

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 adultery ITP spontaneous 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 need combined immunsuppression in ITP, as steroids, high dose invenous immunoglobulins, or in some cases Anti D sera might achieve remission, even if short duration.

Question 17

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 17

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

Question 18

Megaloblastic (pernicious) anemia characteristics:

1) Weak gastric acid output, only after pentagastrin 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 the 4th answer is correct
E) all of the answers are correct

Answer 18

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.

Question 19

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 the 4th answer is correct
E) all of the answers are correct

Answer 19

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.

Question 20

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 the 4th answer is correct
E) all of the answers are correct

Answer 20

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.

Question 21

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 corrected 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 the 4th answer is correct
E) all of the answers are correct

Answer 21

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.

Question 22

True features of agranulocytosis (febrile neutropenia):

1) mostly drug associated
2) mostly idiopathic
3) no erythropoietic 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 the 4th answer is correct
E) all of the answers are correct

Answer 22

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

Question 23

Characteristics of FVII deficiency:

1) rare condition, not inducing bleeding events
2) rare condition with severe bleeding
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 the 4th answer is correct
E) all of the answers are correct

Answer 23

C) 2nd and 4th answers are correct
EXPLANATION
Factor VII deficiency carries high bleeding risk, including gastrointestinal tract as well. It is characterized by isolated spontaneous prothrombin time prolongation (Normal aPTT and TT), and this prolongation could not be corrected by iv vitamin K administration. Right answers are 2 and 4

Question 24

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 the 4th answer is correct
E) all of the answers are correct

Answer 24

C) 2nd and 4th answers are correct
EXPLANATION
Chronic lymphoid leukemia (CLL) sometimes has less duration than other CLL cases. In all 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

Question 25

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 the 4th answer is correct
E) all of the answers are correct

Answer 25

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.

Question 26

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 26

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.

Question 27

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 27

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

Question 28

Heparin complications:

1) Heparin induced thrombocytopenia (HIT) is a mild complication, may induce some easy bruising, injection site reactions
2) HIT is frequently severe 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 derivatives.
5) Heparin may induce cholestatic enzyme 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 28

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.

Question 29

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 29

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