Internal medicine - Hematology (88)

Question 1

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 1

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

Question 2

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 2

ANSWER
C) activated protein C resistence/Leiden-mutation

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

Question 3

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 3

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.

Question 4

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 4

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

Question 5

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 5

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

Question 6

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 6

ANSWER
C) gallstone disease

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

Question 7

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 7

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.

Question 8

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 8

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.

Question 9

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 9

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.

Question 10

INT - 13.11
The most probable background of hypochromic microcyctic anemia:

A) pernicious anemia

B) anemia sideropenica

C) hyperthyreodism

D) agranulocytosis

Answer 10

ANSWER
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

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 11

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

Question 12

INT - 13.13
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

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.

Question 13

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 13

ANSWER
C) 12 hours

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

Question 14

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 14

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

Question 15

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 15

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.

Question 16

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 16

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.

Question 17

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 17

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

Question 18

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 18

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.

Question 19

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 19

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.

Question 20

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 20

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.

Question 21

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 21

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.

Question 22

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 22

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

Question 23

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 23

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

Question 24

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 24

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.

Question 25

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 25

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.

Question 26

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 26

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.

Question 27

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 27

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

Question 28

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 28

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.

Question 29

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 29

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

Question 30

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 30

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

Question 31

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 31

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.

Question 32

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 32

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.

Question 33

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 33

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.

Question 34

INT - 13.39
Acute DIC characteristics:

1) thrombocytopenia

2) normal platelet count

3) shortened PT/aPTT

4) prolonged PT/aPTT

5) hypofibrinogenaemia

6) elevated antithrombin

A) 1st, 4th and 5th answers are correct

B) 2nd, 3rd and 6th answers are correct

Answer 34

ANSWER
A) 1st, 4th and 5th answers are correct

EXPLANATION
Acut DIC is typical consumptional coagulopathy, reducing platelet counts and clotting factor levels. So1,4,5 are correct

Question 35

INT - 13.40
The features of Chronic DIC are:

1) prolonged aPTT

2) slightly shortened aPTT

3) thrombotic events

4) severe bleedings

5) hypofibrinogenaemia

6) elevated D-dimer

7) severe thrombocytopenia

A) 1st and 4th answers are correct

B) 1st, 5th and 7th answers are correct

C) 2nd, 3rd and 6th answers are correct

Answer 35

ANSWER
C) 2nd, 3rd and 6th answers are correct

EXPLANATION
Chronic DIC is a typical procoagulant condition, with thrombotic events, D-dimer positivity, shorter clotting times, so 2,3,6 are the good selection.

Question 36

INT - 13.41
Typical backgrounds of acute DIC:

1) thrombotic thrombocytopenic purpura acute DIC is always present

2) major cranial trauma

3) intrauterin fetal death

4) prostatic cancer

5) Romano–Ward-syndrome

6) multiple myeloma

7) acute promyelocytic leukemia

A) 1st and 5th answers are correct

B) 2nd, 3rd, 4th and 7th answers are correct

C) 5th and 6th answers are correct

Answer 36

ANSWER
B) 2nd, 3rd, 4th and 7th answers are correct

EXPLANATION
In thrombotic thrombocytopenic purpura DIC is extremely rare event. Romano ward syndroma is congenitlal QT prolongation. DIC is unusual in myeloma. Correct responses are 2,3,4,7

Question 37

INT - 13.42
FXIII deficiency typically cause

1) severe bleeding tendency

2) predisposing to thrombosis

3) prolonged bleeding time

4) repeated spontanous abortions

5) increased keloid scars at surgical wounds

6) inreased likelyhood of glaucoma

A) 1st, 4th and 5th answers are correct

B) 2nd., 3rd and 6th answers are correct

Answer 37

ANSWER
A) 1st, 4th and 5th answers are correct

EXPLANATION
FXII defiency induces severe bleedings, spontaneous abortions, and interestingly irregular keloid scar wounds after surgery. Good responses are 1,4,5

Question 38

INT - 13.43
Characteristics of chronic myeloid leukemia

1) survival without specific treatment is around 5 years

2) survival without specific treatment is around 12 years

3) untreated cases average survival is 1 years

4) Cromosome 12 trisomy is a hallmark finding

5) 15,19 translocation is typical

6) By definition Philadelphia chromosome and bcr/abl rearrangerment is present

7) A chronic, accelerated, and blastic phase typicallly develops without specific treatment

8) the disease remains in chronic phase during the entire clinical course

9) blastic phase is a typical finding in early accelerated cases, but very uncommon with early chronic phase cases

A) 1st, 6th and 7th answers are correct

B) 2nd, 4th and 8th answers are correct

C) 3rd, 5th and 9th answers are correct

Answer 38

ANSWER
A) 1st, 6th and 7th answers are correct

EXPLANATION
Chronic myeloid leukemia by definition Philadelphia positive condition, average spontaneous survival is 5 years. It has three phases. chronic, accelerated, and blastic phase. All the other statements are incorrect. Correct 1,6,7

Question 39

INT - 13.44
Typical features of chronic myeloid leukemia

1) Modest splenomegaly

2) Major splenomegaly

3) The entire granulocyte maturation stages cells are represented in peripheral blood

4) Only blasts and matured neutrophils can be seen in peripheral blood smears, intermediary forms are absent

5) Imatinib or second third generation tyrosike kinase inhibitors achieve a complete haematological, cytogenetic and even molecular remission in the majoritiy of chronic phase patients, resulting in huge survival benefit

6) Optimal treatment of chronic phase is prednisolon + cyclophosphamid

A) 1st, 4th and 6th answers are correct

B) 2nd, 3rd and 5th answers are correct

Answer 39

ANSWER
B) 2nd, 3rd and 5th answers are correct

EXPLANATION
In chronic myeloid leukemia remarkable splenomegaly is frequent. You see the entire granulocyte maturation inventory in the peripheral blood. Tirosinkinase inhibitors achieve in most cases hematological and cytogenetical remission, molecular responses, and very good survival. Prednisolon + cyclophosphamide are used in some vasculitis or oaccasionnaly against lymphoproliferation. Correct answers 2,3,5

Question 40

INT - 13.45
Reactive leukocytosis might be expected in:

1) Bacterial bronchopneumonia

2) B virus hepatitis

3) Monoucleosis infectiosa

4) Exsiccosis

5) Anaemia perniciosa

6) Cholecystitis acuta

A) 1st, 2nd and 5th answers are correct

B) 1st, 5th and 6th answers are correct

C) 1st, 3rd, 4th and 6th answers are correct

Answer 40

ANSWER
C) 1st, 3rd, 4th and 6th answers are correct

EXPLANATION
Leukopenia is typical in pernicious anemia and viral hepatitis. Mononucleosis and the other conditions may associate with leukocytosis, Correct, 1,3,4,6

Question 41

INT - 13.46
Leukopenia might be expected in:

1) hyperthyreodism

2) purulent meningitis

3) abscedent pneumonia

4) AIDS premanifest stage

5) acute viral hepatitis

6) autoimmune diseases

7) endocarditis infectiva

A) 1st, 2nd and 6th answers are correct

B) 2nd, 6th and 7th answers are correct

C) 1st, 4th, 5th and 6th answers are correct

Answer 41

ANSWER
C) 1st, 4th, 5th and 6th answers are correct

EXPLANATION
Mononucleosis might associate with leukocytosis. Elevated white bloood count is typical in purulent meningitis, pulmonary abscess, infective endocarditis. Good selection 1,4,5,6

Question 42

INT - 13.47
Eosinophilia might be expected in:

1) Churg-Strauss syndrome
2) Loeffler-endocarditis
3) sphaerocytosis
4) chronic myeloid leukemia, accelerated phase
5) plasmocytoma
6) aplastic anemia

A) 1st, 3rd and 5th answers are correct
B) 1st, 2nd and 4th answers are correct
C) 2nd, 3rd and 5th answers are correct
D) 3rd, 5th and 6th answers are correct

Answer 42

ANSWER
B) 1st, 2nd and 4th answers are correct
EXPLANATION
In aplastic anemia no eosinophilcells are seen, along with other normal blood cells. In plasmocytoma os spherocytosis eosinophilic couunts are normal. So 1 2 4 are the good responses

Question 43

INT - 13.48
Bleeding time will be prolonged

1) ITP (immun thrombocytopenic purpura)

2) aspirin treatment

3) Coumarol administration

4) haemophilia A

5) haemophilia B

6) von Willebrand-disease

A) 1st, 2nd and 6th answers are correct

B) 3rd, 4th and 5th answers are correct

Answer 43

ANSWER
A) 1st, 2nd and 6th answers are correct

EXPLANATION
In coagulation factor deficiencies bleeding time are normal. In thrombocytopenia, altered platelet function and aspirin use prolonged bleeding time observed.

Question 44

INT - 13.49
Clotting time will prolonged

1) Glanzmann-thrombasthenia

2) aspirin user

3) coumarol treatment

4) haemophilia A

5) Willebrand-disease

6) unfractionated heparin therapy

A) 1st and 2nd answers are correct

B) 2nd and 4th answers are correct

C) 3rd, 4th and 6th answers are correct

Answer 44

ANSWER
C) 3rd, 4th and 6th answers are correct

EXPLANATION
Thromcytopathy does not prolong coagulation times, whereas factor deficiency and coumarol does.

Question 45

INT - 13.50
aPTT will be prolonged

1) immun thrombocytopenic purpura

2) acute DIC

3) Unfractionated heparin therapy

4) Low molecular weight heparin administration

5) lupus-anticoagulant case

6) clopidogrel therapy

7) Antithrombin deficiency

A) 1st and 4th answers are correct

B) 2nd, 3rd and 5th answers are correct

C) 6th and 7th answers are correct

Answer 45

ANSWER
B) 2nd, 3rd and 5th answers are correct

EXPLANATION
In acute DIC aPTT is prolonged due to factor consumption, while unfractionatd heparin does it by antithrombin anti Xa activities. In lupus anticoagulat (antiphospholipid syndrome aPTT is prolonged in vitro (complexes are blocking catalytic surfaces to the reaction) whereas in vivo more thrombotic even might develop. LMWHS does not really prolong aPTT, due their specific anti Xa actity, aPTT is also not modified either by Leiden mutation or or antiplatelet durg ticlopidin

Question 46

INT - 13.51
Typical findings in hemolysis:

1) direct bilirubin elevated

2) indirect bilirubin elevated

3) ubg reaction increased in urine

4) bilirubin positivity in urine

5) high reticulocyte count

6) low reticulocyte count

A) 1st and 4th answers are correct

B) 4th and 6th answers are correct

C) 1st, 4th and 6th answers are correct

D) 2nd, 3rd and 5th answers are correct

Answer 46

ANSWER
D) 2nd, 3rd and 5th answers are correct

EXPLANATION
Correct responses are 2,3,5. In hemolysis indirect bilirubin released to circulation and not excreted via kidneys. This also results in more direct bilirubin excretion into the intestine, where it is transformed to urobilinogen, which recirculates, and more urobilinogen will appear in the urine. Due to marrow compensatory activity, more red blood cells will be produced, resulting in elevated reticulocyte counts. Correct selection 2,3,5

Question 47

INT - 13.52
Hemolysis suggestive findings:

1) Coombs-positivity

2) pruritus

3) hypocellular hemopoiesis

4) elevated LDH activity

5) high GOT/GPT ratio

6) pleiochrom, dark stool

A) 1st, 4th and 6th answers are correct

B) 2nd, 3rd and 5th answers are correct

C) 2nd and 3rd answers are correct

D) 3rd and 5th answers are correct

Answer 47

ANSWER
A) 1st, 4th and 6th answers are correct

EXPLANATION
Coombs positivity is frequent finding in immunohemolytic anemias. Red cell LDH release results in elevated LDH activity. GOT/GPT ratio is intact in hemolysis, this may serve as a marker of alcoholic abuse. Stool will be dark due to inreased bilirubin content. Itching is not appearing in hemolytic conditions.

Question 48

INT - 13.53
Diseases associted with hemolysis:

1) plasmocytoma

2) acute ITP (immun thrombocytopenic purpura)

3) paroxysmal nocturnal haemoglobinuria

4) aplastic anemia

5) sphaerocytosis

6) Dubin–Johnson-syndrome

7) anemia perniciosa

8) thrombotic thrombocytopenic purpura/Moschcowitz-syndrome

9) Glanzmann-thrombasthenia

A) 1st and 4th answers are correct

B) 4th, 6th and 9th answers are correct

C) 3rd, 5th, 7th and 8th answers are correct

Answer 48

ANSWER
C) 3rd, 5th, 7th and 8th answers are correct

EXPLANATION
Haemolysis is a hallmark finding in paroxysmal nocturnal hemoglobinuria. Frequent, but moderate hemolysis is characteristic to megaloblastic anemias. Non immune hemolysis is practically always present in TTP/HUS thrombotic microangiopathy. Plasmocytoma does not provoke hemolytic events. Dubin-Johnson is a congenital hepatic birlirubin metabolic disease, Glanzmann thrombasthenia is hereditary platelet function abnormality. Spherocytosis is a typical hemolysis inducing abnormality, doe to reduced osmotic resistance of red blood cells. Correct answer:3 5 7 8

Question 49

INT - 13.54
Two of the following conditions may be associated with hemolysis:

1) ulcerative colitis

2) Crohn-disease

3) blood group mismatched blood transfusion

4) severe preecclampsia (HELLP)

5) myelofibrosis

6) polycythaemia vera

7) thrombocythaemia essentialis

A) 1st and 7th answers are correct

B) 2nd and 6th answers are correct

C) 3rd and 4th answers are correct

D) 5th and 6th answers are correct

Answer 49

ANSWER
C) 3rd and 4th answers are correct

EXPLANATION
Severe hemolysis is induced incompatible blood transfusion and also praeecclapmpsia-HELLP syndrome. Whereas you do not see this in inflammatory bowel diseases, myelofibrosis polycythemia, thrombocythemia.

Question 50

INT - 13.55
Identify chronic myeloproliferative neoplasm among the listed conditions:

1) hemochromatosis

2) Waldenström-macroglobuliemia

3) polycythaemia vera

4) myelofibrosis

5) megaloblastic anemia

6) chronic myeloid leukemia

7) thombocythemia essentialis

8) mycosis fungoides

9) Sezary-syndrome

A) 1st and 2nd answers are correct

B) 3rd, 4th, 6th and 7th answers are correct

C) 5th, 8th and 9th answers are correct

Answer 50

ANSWER
B) 3rd, 4th, 6th and 7th answers are correct

EXPLANATION
By definition chronic myeloproliferativ neoplasms chronic clonal proliferative disorders of myeloid (non lymphoid) cell lines, with transformatory potential into myelofibrosis and sometimes into acute leukemia.

Question 51

INT - 13.56
Macrocytic anemia can be found in:

1) hyperthyroidism

2) anemia perniciosa

3) anemia sideropenica

4) hypothyroidism

5) spherocytosis

A) 1st and 2nd answers are correct

B) 2nd and 4th answers are correct

C) 3th and 5th answers are correct

Answer 51

ANSWER
B) 2nd and 4th answers are correct

EXPLANATION
Correct 2 and 4. Macrocytosis is typical in chronic, especially alcoholic liver disease and megaloblastic anemia. Anemia is rare in hyperthreoidism, whereas anemia in iron deficiency or spherocytosis comes reduced red blood cell size. Correct 2 4.

Question 52

INT - 13.57
Normocytic anemia should be expected

1) chronic iron deficiency

2) B12-malabsorption

3) folic acid impaired absorption

4) recent major bleeding

5) some cancers

A) 1st and 2nd answers are correct

B) 2nd and 3th answers are correct

C) 4th and 5th answers are correct

Answer 52

ANSWER
C) 4th and 5th answers are correct

EXPLANATION
Iron deficiency comes with microcytosis – while B12 and folate deficiency with macrocytic anemia. Correct 4 5

Question 53

INT - 13.58
Severe anemia signs and complaints

1) central cyanosis

2) peripheral cyanosis

3) accidental ejection type systolic murmur

4) tinnitus

5) dizzyness

6) high fever

7) dyspnea

8) hypotension

9) vomiting

A) 1st, 2nd, 6th and 8th answers are correct

B) 2nd, 6th, 8th and 9th answers are correct

C) 3rd, 4th, 5th and 7th answers are correct

Answer 53

ANSWER
C) 3rd, 4th, 5th and 7th answers are correct

EXPLANATION
Cyanosis is induced by hypoxic conditions or polyglobulia, not by anemia. Pyrosis, increased gastric output, low blood pressure, vomiting and fever are not infleunced by anemia. Correct 3 4 5 7

Question 54

INT - 13.59
Immuno-hemolytic characteristic findings

1) Coombs positivity

2) GOT elevated

3) alcaline phosphatase elevated

4) LDH elevated

5) splenectomy helps in 50%

6) splenectomy helps in most instances

7) a splenectomy clearly useless

A) 1st, 4th and 5th answers are correct

B) 3rd and 7th answers are correct

C) 3rd, 6th and 7th answers are correct

Answer 54

ANSWER
A) 1st, 4th and 5th answers are correct

EXPLANATION
Coombs is positive in immuohemolysis. LDH activiy is high in all types of hemolysis. GOT and alcaline phosphatase are not influenced. Splenectomy might be fffective in 50% of refractory immunohemolytic patients. Correct 1 4 5

Question 55

INT - 13.60
Which statements are correct in diffuse large B cell lymphoma?

1) indolent lymphoma

2) aggressive lymphoma

3) antiCD20 based immunochemotherapy achieves definitive cure in substantial part of patients

4) antiCD20 based immunochemotherapy should be applied, but it never achieves final cure

5) In refractory/relapsed cases high dose chemotherapy/autologous transplant should be performed

6) Diffuse large B cell lymphoma is CD20 negative, so anti CD20 therapy is not applicable

A) 2nd, 3th and 5th answers are correct

B) 3rd and 5th answers are correct

C) 4th and 5th answers are correct

D) 2nd and 6th answers are correct

Answer 55

ANSWER
A) 2nd, 3th and 5th answers are correct

EXPLANATION
Diffuse large B cell lymphoma is a so called prototype of aggressive lymphomas. They are CD20 positive, anti CD20 based immunochemotherapy may achive final cure in large proportion of patients. Refratory or relapsed cases should receive anti CD20 based salvage therapy combinations and autologoius stem cell transplantation.

Question 56

INT - 13.62
You can find elevated iron plasma levels in:

1) myeloma multiplex

2) hemochromatosis

3) Willebrand disease in females

4) hemolytic crisis

5) aplastic anemia

6) agranulocytosis

A) 1st and 6th answers are correct

B) 1st, 5th and 6th answers are correct

C) 2nd and 4th answers are correct

Answer 56

ANSWER
C) 2nd and 4th answers are correct

EXPLANATION
Iron levels are high in hemochromatosis and in hemolytic conditions (due to red blood cell disruption).Iron metabolism is intact in myeloma, aplastic anemia, agranulocytosis and Willebrand disease.

Question 57

INT - 13.65
Feasible methods to detect empty iron stores

1) serum iron level

2) Prussian blue stain, bone marrow

3) Shilling-test

4) Ham-test

5) serum-ferritin

6) β2-microglobulin

7) PAS-reaction, bone marrow

A) 1st, 3rd and 7th answers are correct

B) 3rd, 4th and 6th answers are correct

C) 2nd and 5th answers are correct

Answer 57

ANSWER
C) 2nd and 5th answers are correct

EXPLANATION
Serum iron levels are not reflecting iron stores saturation. Shilling test is an old approach to examine B12 absorption. HAM test is a traditional screening method paroxysmal nocturnal hemoglobinuria. B2 microglobulin may reflect B cell proliferation and immuno reactions. Bone marrow PAS stain is a traditional bone marrow cytochemistry in acute lymphoid leukemia

Question 58

INT - 13.68
Important viruses in aplastic anemia etiopathogenesis:

1) Epstein–Barr-virus

2) cytomegalovirus

3) parvovirus

4) hepatitis A virus

5) HIV virus

6) hepatitis B, C virus

7) mumps virus

A) 1st, 2nd and 4th answers are correct

B) 2nd, 4th and 7th answers are correct

C) 3rd, 5th and 6th answers are correct

Answer 58

ANSWER
C) 3rd, 5th and 6th answers are correct

EXPLANATION
Viruses mentioned at items 3, 5, 6 are really considered pathogenic in some aplastic anemia cases, while the others are not.

Question 59

INT - 13.69
Select correct features of agranulocytosis (febrile neutropenia)
1) Bone marrow aplasia, involving all cell lines

2) Bone marrow: Large number of early myeloid precursors, blocked in maturation, other cell lines are preserved

3) Normal hemopoiesis in bone marrow

4) Agranocytosis improves with the cessation of provocative drug.

5) This improvement can be slightly quicker with bone marrow cytokin administration

6) Agranulocytosis is not a drug induced condition, so cessation of previous drugs is unnecessary.

7) Large dose fresh frozen plasma administration helps agranulocytotic patients

A) 1st, 6nd and 7th answers are correct

B) 2nd, 4th and 5th answers are correct

C) 3rd, 6th and 7th answers are correct

Answer 59

ANSWER
B) 2nd, 4th and 5th answers are correct

EXPLANATION
In agranulocytosis (febrile neutropenia) you find isolated granulocyopenia, with normal platelet and red blood cell counts. One sees early myeloid cell maturation block in bone marrow. Almost all adultory cases are drug induced, so immediate cessation of culprit drug is essential, which is follwed by restoration of granulopoesis, which might be slightly speed up by addition of GCSF. Plasma administration is clearly unnecessary. So item B contains the good response combination

Question 60

INT - 13.70
You should apply perioperative anticoagulant prophylaxis:

1) all surgery lasting more than 20 minutes

2) 45 minutes or longer operation, even if laparoscopic

3) all cases with general anaesthesia

4) neoplasm surgery

5) hip joint or fracture surgery

6) all upper limb bone surgery

A) 1st, 3rd and 6th answers are correct

B) 2nd, 4nd and 5th answers are correct

Answer 60

ANSWER
B) 2nd, 4nd and 5th answers are correct

EXPLANATION
2, 4, 5 are the musts, according to standard recommendations and guidelines. Short surgery or short general anaesthesia is no an obligatory indication

Question 61

INT - 13.71
Correct statements in mantle cell lymphoma:

1) Dense CD 20+ lymphoma so anti CD20 based intensive immunochemotherapy should be applied

2) CD20 negative, so anti CD 20 therapy is not necessary

3) Autologous transplant should be performed in first remission

4) Indolent course is frequent in which watch and wait approach is enough

5) indolent lymphoma

6) Aggressive course is typical in vast majority

A) 1st, 3rd and 6th answers are correct

B) 2nd, 4th and 5th answers are correct

Answer 61

ANSWER
A) 1st, 3rd and 6th answers are correct

EXPLANATION
Correct is 1,3,6. as CD20 high density positivity is characteristic in mantle cell lymphoma. based upon this first line therapy is antiCD20 based intensive immunochemotherapy. In fisrt remission consolidation with autologous stem cell transplant is strongly indicated. Vast majority of Mantle cell lymphoma cases run aggressive course

Question 62

INT - 13.72
Coumarol overdose induced major bleeding can be stopped by only three of the choices:

1) protamine sulphate

2) low molecular weight heparin

3) ε-aminocaproic acid

4) fresh frozen plasma

5) DDAVP

6) Novoseven (rFVIIa) iv.

7) prothrombin complex concentrate

A) 1st, 2nd and 5th answers are correct

B) 2nd, 3rd and 4th answers are correct

C) 4th, 6th and 7th answers are correct

Answer 62

ANSWER
C) 4th, 6th and 7th answers are correct

EXPLANATION
Protamin is antidote for unfractionated heparin, so it is not convenient in this condition. Similarly LMWH is also an anticoagulant, which is contraindicated in bleeding. DDAVP infusin might be used in Willebrand disease. 4, 6, 7 are are the correct approaches.

Question 63

INT - 13.73
Which is the most likely disease?

A 16 years old girl admitted to your ward, looks really sick. History: no meaningful previous event, she had regular sport activity, but 8 days ago she had transient mild fever and diarrhoea for 2 days. Last day she had fever again, headache, dizziness, mild confusion, she could not even recognise her sister. Small petechiae and suffusiones appeared. You observe somnolence, mild jaundice. No meningeal signs. Liver, spleen, lymphnodes are normal sized. Platelet count 10 G/L, WBC 15 G/L, urea 11 mmol/l, hemostasis screening tests are normal.
A) acute myeloid leukemia

B) acute lymphoid leukemia

C) acute immun thrombocytopenic purpura (ITP)

D) Thrombotic mikroangiopathia, HUS (hemolytic uremic syndrome)

E) acute víral hepatitis

F) acute DIC

Answer 63

ANSWER
D) Thrombotic mikroangiopathia, HUS (hemolytic uremic syndrome)

EXPLANATION
Acute myeloid or lymphoid leukemias are not typically associated with cognitive dysfunctions or previous diarrhoea episodes. In acute viral hepatitis platelet counts are usually normal. Confusion is very rare in acute DIC early stage, and there are severe bleedings along with laboratory signs of consumption coagulopathy. This clinical presentation presentation is typical to thrombotic microangiopathy HUS or TTP. So correct answer is D.

Question 64

INT - 13.74
Which are the most appropriate examination in this case to prove suggested diagnosis

A 16 years old girl admitted to your ward, looks really sick. History: no meaningful previous event, she had regular sport activity, but 8 days ago she had transient mild fever and diarrhoea for 2 days. Last day she had fever again, headache, dizziness, mild confusion, she could not even recognise her sister. Small petechiae and suffusiones appeared. You observe somnolence, mild jaundice. No meningeal signs. Liver, spleen, lymphnodes are normal sized. Platelet count 10 G/L, WBC 15 G/L, urea 11 mmol/l, hemostasis screening tests are normal.
1) Bone marrow – myeloblast accumulation

2) Bone marrow – lymphoblast accumulation

3) Bone marrow – accumulation

4) GOT-/GPT

5) HbsAg screening

6) LDH-activity

7) peripheral blood smear, leukocytosis analysis

8) peripheral blood smear, lymphoblast search

9) peripheral blood smear, fragmentocyte (fragmented sred blood cells) screen

10) Platelet surface immunglobulin detection

11) kidney function analysis

A) 1st, 4th and 11th answers are correct

B) 2nd, 5th and 10th answers are correct

C) 6th, 9th and 11th answers are correct

D) 7th, 8th and 10th answers are correct

Answer 64

ANSWER
C) 6th, 9th and 11th answers are correct

EXPLANATION
In HUS one may find excessive number of bone marrow megakaryocytes (same as in some other conditions, i.e. ITP) so this examination is not really indicated. Elevated LDH, Fragmented red blood cells in peripheral blood smears, modest renal impairment should be searched and detected as simple but important diagnostic tools. So correct is 6, 9, 11. all the other tests are useless in confirming your diagnosis.

Question 65

INT - 13.75
Which are the most useful disease specific diagnostic tools

A 16 years old girl admitted to your ward, looks really sick. History: no meaningful previous event, she had regular sport activity, but 8 days ago she had transient mild fever and diarrhoea for 2 days. Last day she had fever again, headache, dizziness, mild confusion, she could not even recognise her sister. Small petechiae and suffusiones appeared. You observe somnolence, mild jaundice. No meningeal signs. Liver, spleen, lymphnodes are normal sized. Platelet count 10 G/L, WBC 15 G/L, urea 11 mmol/l, hemostasis screening tests are normal.
1) Bone marrrow myeloblast flow cytometry

2) t15,17 translocation, suggesting acute promyelocytic leukemia, to indicate retinooid therapy

3) Durable increment of platelet counts after platelet transfusion, a sin ITP foreign platelets are not affected by antibodies

4) test if patient plasma provokes platelet aggregation in control specimen

5) verotoxin ELISA

6) ADAM TS13 activity or complementactivation measurement

7) TFPI measurement (tissue Factor inhibitor)
A) 1st and 7th answers are correct

B) 5th and 6th answers are correct

C) 3rd and 7th answers are correct

D) 4th and 5th answers are correct

E) all of the answers are correct

Answer 65

ANSWER
B) 5th and 6th answers are correct

EXPLANATION
HUS plasma might provoke healthy platelet clumping phenomenon, but this is uncertain and not used as specific test. Verotoxin ELISA seemes to be important in post diarrhoea HUS cases. More recently we understood that the most common background is ADAMTS13 abnormality, and there are cases with complement system abnormality. All other options are irrelevant. So the correct selection is 5, 6.

Question 66

INT - 13.76
Which therapeutic approach is the most appropriate

A 16 years old girl admitted to your ward, looks really sick. History: no meaningful previous event, she had regular sport activity, but 8 days ago she had transient mild fever and diarrhoea for 2 days. Last day she had fever again, headache, dizziness, mild confusion, she could not even recognise her sister. Small petechiae and suffusiones appeared. You observe somnolence, mild jaundice. No meningeal signs. Liver, spleen, lymphnodes are normal sized. Platelet count 10 G/L, WBC 15 G/L, urea 11 mmol/l, hemostasis screening tests are normal.
A) AML induction chemoterapy

B) ALL induction chemotherapy

C) retinoic acid administration

D) Antibiotics

E) high dose plasma administration/plasmaapheresis

F) aspirin

Answer 66

ANSWER
E) high dose plasma administration/plasmaapheresis

EXPLANATION
Large volume plasma exhcange therapy untill improvement has crucial importance

Question 67

INT - 13.77
What interventions are contraindicated in the acute phase of this disease?

A 16 years old girl admitted to your ward, looks really sick. History: no meaningful previous event, she had regular sport activity, but 8 days ago she had transient mild fever and diarrhoea for 2 days. Last day she had fever again, headache, dizziness, mild confusion, she could not even recognise her sister. Small petechiae and suffusiones appeared. You observe somnolence, mild jaundice. No meningeal signs. Liver, spleen, lymphnodes are normal sized. Platelet count 10 G/L, WBC 15 G/L, urea 11 mmol/l, hemostasis screening tests are normal.

1) thrombocyte transfusion

2) colony stimulators (GCSF, GM-CSF)

3) prednisolon

4) antibiotics

5) diuretics

6) clopidogrel

7) aspirin

A) 1st, 6th and 7th answers are correct

B) 2nd, 6th and 7th answers are correct

C) 3rd, 4th and 5th answers are correct

Answer 67

ANSWER
A) 1st, 6th and 7th answers are correct

EXPLANATION
Steroids, antibiotic, or some diuretics might be useful or recemmended in TTP/HUS. Platelet transfusion must not be applied. Transfusions, cytokines are unnecessary. Platelet inhibitors are contraindicated in the acute period of the disease So ceorrect selection 1,6,7

Question 68

INT - 13.78
Which more recent therapy can also be helpful

A 16 years old girl admitted to your ward, looks really sick. History: no meaningful previous event, she had regular sport activity, but 8 days ago she had transient mild fever and diarrhoea for 2 days. Last day she had fever again, headache, dizziness, mild confusion, she could not even recognise her sister. Small petechiae and suffusiones appeared. You observe somnolence, mild jaundice. No meningeal signs. Liver, spleen, lymphnodes are normal sized. Platelet count 10 G/L, WBC 15 G/L, urea 11 mmol/l, hemostasis screening tests are normal.
A) heparin

B) autologous bone marrow transplantation

C) allogeneous bone marrow transplantation

D) iv. anti cd20 monoclonal antibody

E) high dose interferon

Answer 68

ANSWER
D) iv. anti cd20 monoclonal antibody

EXPLANATION
In refractory cases anti CD20 monoclonal antibody may be effective. Correct D

Question 69

INT - 13.79
With adequate diagnosis and therapy what is the correct prognosis

A 16 years old girl admitted to your ward, looks really sick. History: no meaningful previous event, she had regular sport activity, but 8 days ago she had transient mild fever and diarrhoea for 2 days. Last day she had fever again, headache, dizziness, mild confusion, she could not even recognise her sister. Small petechiae and suffusiones appeared. You observe somnolence, mild jaundice. No meningeal signs. Liver, spleen, lymphnodes are normal sized. Platelet count 10 G/L, WBC 15 G/L, urea 11 mmol/l, hemostasis screening tests are normal.

A) 90% mortality remains unchanged- in spite of therapeutic efforts

B) 5 years survival 15–30%

C) with steroids+splenectomy you can achieve definitive cure, sometimes with residual easy bruising

D) defintive cure rate is 90–95%, some cases meay have recidive episodes

E) Spontaneous improvement 80%, with treatment it is almost 100%

Answer 69

ANSWER
D) defintive cure rate is 90–95%, some cases meay have recidive episodes

EXPLANATION
Plasma administration/apheresis based therapy achieves more than 90% cure, although sometimes recidives may develop. Correct D

Question 70

INT - 13.80
This disease might have recidives more likely in

A 16 years old girl admitted to your ward, looks really sick. History: no meaningful previous event, she had regular sport activity, but 8 days ago she had transient mild fever and diarrhoea for 2 days. Last day she had fever again, headache, dizziness, mild confusion, she could not even recognise her sister. Small petechiae and suffusiones appeared. You observe somnolence, mild jaundice. No meningeal signs. Liver, spleen, lymphnodes are normal sized. Platelet count 10 G/L, WBC 15 G/L, urea 11 mmol/l, hemostasis screening tests are normal.
A) pneumococcus-pneumonia

B) Philadelphia-chromosome anomaly present

C) lg H gene rearrangement is persistent

D) pregnancy

E) aspirin use

F) Taking coumarols

Answer 70

ANSWER
D) pregnancy

EXPLANATION
Pregnancy and hormonal anticoncipients may promote recidive episodes, all the others are not. Aspirin may even prevent relapses, Correct D

Question 71

INT - 13.81
Dominant pathogentic factor in this disease

A 16 years old girl admitted to your ward, looks really sick. History: no meaningful previous event, she had regular sport activity, but 8 days ago she had transient mild fever and diarrhoea for 2 days. Last day she had fever again, headache, dizziness, mild confusion, she could not even recognise her sister. Small petechiae and suffusiones appeared. You observe somnolence, mild jaundice. No meningeal signs. Liver, spleen, lymphnodes are normal sized. Platelet count 10 G/L, WBC 15 G/L, urea 11 mmol/l, hemostasis screening tests are normal.
A) retinoin receptor anomaly

B) previous alkylator therapy

C) previous irradiation

D) hypertension

E) ADAMTS13 or complement alteration

F) multidrug-resistane

Answer 71

ANSWER
E) ADAMTS13 or complement alteration

EXPLANATION
ADAMTS13 blocking antibody or deficiency is most common background of TTP/HUS. Correct E, all other options are irreleveant

Question 72

INT - 13.82
Which intervention is strictly contraindicated in this disease

A 16 years old girl admitted to your ward, looks really sick. History: no meaningful previous event, she had regular sport activity, but 8 days ago she had transient mild fever and diarrhoea for 2 days. Last day she had fever again, headache, dizziness, mild confusion, she could not even recognise her sister. Small petechiae and suffusiones appeared. You observe somnolence, mild jaundice. No meningeal signs. Liver, spleen, lymphnodes are normal sized. Platelet count 10 G/L, WBC 15 G/L, urea 11 mmol/l, hemostasis screening tests are normal.
A) Red blood cell-transfusion

B) Platelet transfusion

C) splenectomy

D) colony stimulators, fdue to leukemogenicity

Answer 72

ANSWER
B) Platelet transfusion

EXPLANATION
Platelet transfusion may severely worsen TTP/HUS clinical signs, so it it strictly contraindicated. All other options are rather neutral. Correct B

Question 73

INT - 13.83
What is the most likely diagnosis suspected?

You see a 62 years old male, with cherry sized neck, axillary, inguinal painless lymphadenopathy. Moderate weakness and weightloss. He had some mild fever and upper airway infections recently. Spleen enlarged by 6 cm. White Blood cell count 23 G/L, 76% small matured lymphocyte, other forms are normal. Hemoglobin 105 g/l, platelet count normal. Reduced IgG.
A) lymphogranulomatosis

B) chronic lymphoid leukemia

C) acute lymphoid leukemia

D) chronic myeloid leukemia

E) mononucleosis infectiosa

Answer 73

ANSWER
B) chronic lymphoid leukemia

EXPLANATION
The clinical presentation is a typical case of chronic lymphocytic leukemia (CLL). Characteristic is advanced age, lymphocytosis with seemingly matured small lymphocytes, lymphadenopathy, modest anemia somtimes mild spleen enlargement. So correct is B. In Hodgkin you do not see lymphocytosis or low immunoglobulins. Mononucleosis is the disease of young people. In chronic myeloid leukemia granulocyte cell line accumulation might be observed. In acute lymphoid leukemia lymphoblasts are seen.

Question 74

INT - 13.84
What are the most helpful diagnostic tools in this case

You see a 62 years old male, with cherry sized neck, axillary, inguinal painless lymphadenopathy. Moderate weakness and weightloss. He had some mild fever and upper airway infections recently. Spleen enlarged by 6 cm. White Blood cell count 23 G/L, 76% small matured lymphocyte, other forms are normal. Hemoglobin 105 g/l, platelet count normal. Reduced IgG.
1) lymph node biopsy

2) Gumprecht shadows in peripheral blood smears

3) Bone marrow examination

4) citogenetics screening for Philadelphia-chromosome

5) peripheral blood flowcytometry. CD5/CD19 coexpressed cell count measurement
A) 1st and 3rd answers are correct

B) 2nd and 4th answers are correct

C) 2nd and 5th answers are correct

D) 4th and 5th answers are correct

E) all of the answers are correct

Answer 74

ANSWER
C) 2nd and 5th answers are correct

EXPLANATION
Correct are 2 and 5. The appearance of 5 G/L or more CD19/CD5 coexpressed lymphocytes in peripheral blood is the official diagnostic criterium, Gumprecht shadows are additional markers (but not enough in itself). Paul Bunnel and Philadelphia are used in mononucleosis and CML. You do not need lymph node or bone marrrow biopsy to approve your diagnosis.

Question 75

INT - 13.85
What diagnostic tool is the most useful in this condition to define prognosis

You see a 62 years old male, with cherry sized neck, axillary, inguinal painless lymphadenopathy. Moderate weakness and weightloss. He had some mild fever and upper airway infections recently. Spleen enlarged by 6 cm. White Blood cell count 23 G/L, 76% small matured lymphocyte, other forms are normal. Hemoglobin 105 g/l, platelet count normal. Reduced IgG.
A) Epstein–Barr-víral serology

B) Epstein–Barr-vírus genomic analysis in malignant cells

C) Bcr-Abl rearrangement analysis, even if cytogenetics are negative

D) Philadelphia chomosome detection

E) PAS and acid phosphatase citochemistry

F) peripheral blood FISH analysis

Answer 75

ANSWER
F) peripheral blood FISH analysis

EXPLANATION
Correct selection is F, as FISH information are important factors in prognosis and progression of disease. All the others are not related to CLL.

Question 76

INT - 13.86
What clinical signs, complications you should check and foresee for longer period

You see a 62 years old male, with cherry sized neck, axillary, inguinal painless lymphadenopathy. Moderate weakness and weightloss. He had some mild fever and upper airway infections recently. Spleen enlarged by 6 cm. White Blood cell count 23 G/L, 76% small matured lymphocyte, other forms are normal. Hemoglobin 105 g/l, platelet count normal. Reduced IgG.
1) slowly progressing lymphadenomegaly

2) splenic rupture

3) hepatitis like symptoms

4) myocarditis like symptoms

5) increased likelyhood of infections

6) transformation into accelerated or blastic phase

7) transformation into myelofibrosis

8) After the cure of lymphogranulomatosis secondary cancer might develop

A) 2nd and 4th answers are correct

B) 6th and 7th answers are correct

C) 3rd and 8th answers are correct

D) 1st and 5th answers are correct

Answer 76

ANSWER
D) 1st and 5th answers are correct

EXPLANATION
Slowly progressive lymphadenopathy and increased frequency of infections are the most common characteristics to CLL, so correct is D. Blast transformation occurrs in CML. option 8 might be valid only for Hodgkin patient.

Question 77

INT - 13.87
How much is the average survival of the disease while treating?

You see a 62 years old male, with cherry sized neck, axillary, inguinal painless lymphadenopathy. Moderate weakness and weightloss. He had some mild fever and upper airway infections recently. Spleen enlarged by 6 cm. White Blood cell count 23 G/L, 76% small matured lymphocyte, other forms are normal. Hemoglobin 105 g/l, platelet count normal. Reduced IgG.
A) spontaneous cure in all

B) One or two years in spite of therapeutic efforts

C) Four years chronic phase, followed by one year accelerated phase

D) Fove year survival is 55–60% in Hodgkin lymphoma

E) 8–15 average survivial, however final cure can be achieved even with modern therapies

Answer 77

ANSWER
E) 8–15 average survivial, however final cure can be achieved even with modern therapies

EXPLANATION
In most CLL cases E is the good selection, survival is long but CLL for the time being is still incurable condition. C is valis for CML, D would be lymphogranulomatosis, but survival is not even correctly given for this disease.

Question 78

INT - 13.88
Which alteration carries important prognostic relevance

You see a 62 years old male, with cherry sized neck, axillary, inguinal painless lymphadenopathy. Moderate weakness and weightloss. He had some mild fever and upper airway infections recently. Spleen enlarged by 6 cm. White Blood cell count 23 G/L, 76% small matured lymphocyte, other forms are normal. Hemoglobin 105 g/l, platelet count normal. Reduced IgG.

1) histological subtype, as lymphoid depletion cases run poor prognosis

2) In ALL Philadelphia chromosome means very bad prognosis

3) lymphocyte doubling time shorter than 6 month

4) anemia, thrombocytopenia, medullary infiltration

5) male sex in itself has worse prognosis in Hodgkin

6) In chronic lymphocytic leukemia FISH p53 positivity especaillay bad prognosis

A) 1st and 3rd answers are correct

B) 3rd and 6th answers are correct

C) 4th and 6th answers are correct

Answer 78

ANSWER
B) 3rd and 6th answers are correct

EXPLANATION
B is the good selection, as short cell doubling time or FISH p53 positivity indicates progression or poor prognosis.

Question 79

INT - 13.89
What is the adequate therapy in this case?

You see a 62 years old male, with cherry sized neck, axillary, inguinal painless lymphadenopathy. Moderate weakness and weightloss. He had some mild fever and upper airway infections recently. Spleen enlarged by 6 cm. White Blood cell count 23 G/L, 76% small matured lymphocyte, other forms are normal. Hemoglobin 105 g/l, platelet count normal. Reduced IgG.
A) hydroxyurea first line, may consider interferons which might induce cytogenetic response of Phildelphia abnormality

B) ALL-standard agents: antracyclin, steroid, vincristine, methotrexate polychemotherapy

C) in mononucleosis observation is enough without any intervention mononucleosis

D) COPP (cyclophosphamide + vincristine + procarbazin + prednisolone) protocoll, 6 cycles

E) watch and wait, if progression (quick doubling of white blood cell count, gross lymphadenopathy, B signs) first line should be Fludarabine-Cyclophosphamid-MabThera immunchemotherapy, in elderly, moderately frail patients MabThera+bendamustin

Answer 79

ANSWER
E) watch and wait, if progression (quick doubling of white blood cell count, gross lymphadenopathy, B signs) first line should be Fludarabine-Cyclophosphamid-MabThera immunchemotherapy, in elderly, moderately frail patients MabThera+bendamustin

EXPLANATION
Watch and wait is the best approach in early CLL, therapy is indicated only if cell doubling is shorter than 6 month, lymph node enlargement is quick, progressive or there are B signs. So correct selection is E. All other options are irrelevant in CLL.

Question 80

INT - 13.90
Which 3 alteration may help the most during followup

You see a 62 years old male, with cherry sized neck, axillary, inguinal painless lymphadenopathy. Moderate weakness and weightloss. He had some mild fever and upper airway infections recently. Spleen enlarged by 6 cm. White Blood cell count 23 G/L, 76% small matured lymphocyte, other forms are normal. Hemoglobin 105 g/l, platelet count normal. Reduced IgG.
1) extent of splenomegaly

2) extent and progression of lymphadenomegalia

3) kinetics of white blood cell count increase

4) immunglobulin levels

5) progression of FISH alterations

6) EBV-antibody -titer changes

7) serum-copper levels

A) 1st, 5th and 7th answers are correct

B) 5th, 6th and 7th answers are correct

C) 2nd, 3rd and 5th answers are correct

D) 1st and 5th answers are correct

E) all of the answers are correct

Answer 80

ANSWER
C) 2nd, 3rd and 5th answers are correct

EXPLANATION
CLL progression markers are 2,3, and 5. Cell number kinetics, quick progression of lymphadenopathy are clear indicators, while bad baseline FISH markers, or secondary FISH alterations are informing you about prognosis or even worse prognosis. EBV markers and copper levels are useless in CLL:

Question 81

INT - 13.91
What other examinations should should be performed before starting anticoagulant therapy?

A 26-year-old pregnant lady in 16 weeks of pregnancy arrives to your office. Progressive swelling and pain developed in he right femoral region, four days ago. Pregnancy goes on normally otherwise. Doppler ultrasound reveals newly developed deep vein thrombosis in right femoral vein and popliteal vein. The patient’s mother and sister also had thrombotic episodes.
1) Iv. phlebography, as all types of anticoagulants are extremely dangerous during pregnancy, to ascertain more your diagnosis

2) aPTT

3) fibrinogen level

4) Factor VIII. activity

5) baseline platelet count

6) Bleeding time measurememnt

A) 1st and 3rd answers are correct

B) 2nd and 5th answers are correct

C) 4th and 6th answers are correct

D) 1st and 5th answers are correct

E) all of the answers are correct

Answer 81

ANSWER
B) 2nd and 5th answers are correct

EXPLANATION
Baseline haemostasis screening is indicated to identify potential thrombophilia (e.g. lupus anticoagulant), but more importantly baseline platelet count is necessary to see whether heparin induced thrombocytopenia develops or not. Good selection 2 and 5. Phlebography is not indicated, as invasive and does not provide more information than Color Doppler, which is the gold standard in deep vein thrombosis diagnostics, and you should not expose a pregnant woman to unnecessary radiation. Fibrinogen level and FVIII activity does not carry any importance in this case. Bleeding time is not altered by deep vein thrombosis

Question 82

INT - 13.92
Which anticoagulant is that does not cross placental barrier and can be administered safely during pregnancy?

A 26-year-old pregnant lady in 16 weeks of pregnancy arrives to your office. Progressive swelling and pain developed in he right femoral region, four days ago. Pregnancy goes on normally otherwise. Doppler ultrasound reveals newly developed deep vein thrombosis in right femoral vein and popliteal vein. The patient’s mother and sister also had thrombotic episodes.
A) low molecular weight heparin

B) coumarol derivatives

C) dabigatran

D) rivaroxaban

Answer 82

ANSWER
A) low molecular weight heparin

EXPLANATION
Low molecular weight heparins (LMWH) do not cross placenta, they might be given during pregnancy without any harm to the fetus or mother. Correct A.

Question 83

INT - 13.93
Wich anthrombotic therapy should be selected in this case?

A 26-year-old pregnant lady in 16 weeks of pregnancy arrives to your office. Progressive swelling and pain developed in he right femoral region, four days ago. Pregnancy goes on normally otherwise. Doppler ultrasound reveals newly developed deep vein thrombosis in right femoral vein and popliteal vein. The patient’s mother and sister also had thrombotic episodes.
A) only aspirin can be applied in pregnancy

B) Low molecular weight heparin, therapeutic dose

C) couumarin, as all heparins are contraindicated in pregnant patients

D) thrombolytic treatment

E) surgical thrombectomy

Answer 83

ANSWER
B) Low molecular weight heparin, therapeutic dose

EXPLANATION
Correct is B. LMWH may be administered for thrombotic or thrombophiliac pregnants. coumarols should be avoided, being teratogenic. thrombolytic treatment if possible should not be used during pregnancy (placental damage). In this case thrombolytic approach is not indicated. Surgical thrombectomy does not seems necessary, as well. Aspirin is not a convenient agent for venous thrombos

Question 84

INT - 13.94
Considering all this, what are the feasible options

A 26-year-old pregnant lady in 16 weeks of pregnancy arrives to your office. Progressive swelling and pain developed in the right femoral region, four days ago. Pregnancy goes on normally otherwise. Doppler ultrasound reveals newly developed deep vein thrombosis in the right femoral vein and popliteal vein. The patient’s mother and sister also had thrombotic episodes.
1) aspirin
2) aspirin + coumarol
3) Unfractionated-heparin 1000–1200 U/hour, infusion
4) immobilisation, elastic bandage on the thrombotic leg
5) Low molecular weight heparin twice/day 100–120 U/kg subcutaneously.
6) streptokinase or tissue plasminogen activator
7) pentoxyphyllin

A) 1st and 3rd answers are correct
B) 2nd and 7th answers are correct
C) 4th and 5th answers are correct
D) 3rd and 5th answers are correct
E) all of the answers are correct

Answer 84

ANSWER
C) 4th and 5th answers are correct
EXPLANATION
Good answers 4 and 5, the LMWH doses are in concert with clinical practice

Question 85

INT - 13.95
Identify the most dangerous complication of your therapy:

A 26-year-old pregnant lady in 16 weeks of pregnancy arrives to your office. Progressive swelling and pain developed in he right femoral region, four days ago. Pregnancy goes on normally otherwise. Doppler ultrasound reveals newly developed deep vein thrombosis in right femoral vein and popliteal vein. The patient’s mother and sister also had thrombotic episodes.
A) erosion, gastric bleeding

B) heparin induced thrombocytopenia

C) coumarol-necrosis

D) hypofibrinogenemia

Answer 85

ANSWER
B) heparin induced thrombocytopenia

EXPLANATION
By far the most dangerous side effect is heparin induced thrombocytopenia. Gastric erosions in this setting has no clinical impact. Hypofibrinogenemia is not induced by LMWHs. Coumarol necrosis of course irrelevant

Question 86

INT - 13.96
After the acute thrombotic period which type of anticoagulant prophylaxis should be applied in this case?

A 26-year-old pregnant lady in 16 weeks of pregnancy arrives to your office. Progressive swelling and pain developed in he right femoral region, four days ago. Pregnancy goes on normally otherwise. Doppler ultrasound reveals newly developed deep vein thrombosis in right femoral vein and popliteal vein. The patient’s mother and sister also had thrombotic episodes.
1) Coumarols

2) aspirin

3) Low molecular weight heparin profilactic dose once daily 1

4) elastic bandage

5) prophilaxis is not necessary

6) prohylaxis is dangerous in pregnancy, so not recommended

A) 1st and 3rd answers are correct

B) 3rd and 4th answers are correct

C) 5th and 6th answers are correct

D) 3rd and 5th answers are correct

E) all of the answers are correct

Answer 86

ANSWER
B) 3rd and 4th answers are correct

EXPLANATION
Elastic bandage on the leg along with once daily LMWH is the convenient thrombosis prophylasis during pregnancy. Correct 3 and 4. Coumarol is teratogenic. Aspirin is not able to prevent venous thrombosis. prophylaxis is really necessary and the described form is not dangerous during pregnancy.

Question 87

INT - 13.97
In her case suspicion of thrombophilic condition shoud be an issue. What is the most like hereditary thrombophilia based on statistics?

A 26-year-old pregnant lady in 16 weeks of pregnancy arrives to your office. Progressive swelling and pain developed in he right femoral region, four days ago. Pregnancy goes on normally otherwise. Doppler ultrasound reveals newly developed deep vein thrombosis in right femoral vein and popliteal vein. The patient’s mother and sister also had thrombotic episodes.
A) antithrombin deficiency

B) protein C deficiency

C) activated protein C resistance/F. V. Leiden-mutation

D) protein S deficiency

E) homozygous hyperhomocysteinemia

Answer 87

ANSWER
C) activated protein C resistance/F. V. Leiden-mutation

EXPLANATION
The most frequent hereditary thrombophilia in Europe is Leiden mutation, correct is C.

Question 88

INT - 13.98
So the most common thrombophilia was proved. What prophylactic guideline is recommended in her case?
A 26-year-old pregnant lady in 16 weeks of pregnancy arrives to your office. Progressive swelling and pain developed in he right femoral region, four days ago. Pregnancy goes on normally otherwise. Doppler ultrasound reveals newly developed deep vein thrombosis in right femoral vein and popliteal vein. The patient’s mother and sister also had thrombotic episodes.
A) all cases whoi had once DVT lifelong anticoagulant prophylactic approach recommended.

B) All cases with previous DVT should receive couple of years anticoagulant prophylaxis, after some years may go for cautious dose reduction

C) In all such cases a 6 mont prophylaxis is enough

D) As all prophylactic efforts are ineffective, their use is not recommended

Answer 88

ANSWER
B) All cases with previous DVT should receive couple of years anticoagulant prophylaxis, after some years may go for cautious dose reduction

EXPLANATION
The guidelines indicate anticoagulant prophylaxis in Leiden mutation after the first thrombotic event. Thrombosis free Leiden mutants should not receive that. After a thrombotic event with Leiden mutation anticoagulant prophylaxis is indicated for some years, maybe with tapering dose later on.