Hematology

Question 1

The other major natural cobalamin that is the form in human plasma and in cell cytoplasm.

Answer 1

methylcobalamin,

Question 2

*It is now thought that metformin lowers serum vitamin B12 level by lowering thelevel of

Answer 2

TC I

Question 3

The goal of therapy in IDA is to provide shoes of at least

Answer 3

0.5 to 1gram of IRON

sustained tx 6-12months needed to achieve this

Question 4

True or false:

DIC is diagnosed in almost one-half of pregnant women with abruptio placentae or with amniotic fluid embolism.

Answer 4

True

Question 5

is a severe form of DIC resulting from thrombosis Of extensive areas of the skin; it affects predominantly young children following viral or bacterial infection, particularly those with inherited or acquired hypercoagulability due to deficiencies of the components of the protein C pathway

Answer 5

Purpura fulminans

Question 6

The central mechanism of DIC is the

Answer 6

uncontrolled generation of thrombin by exposure of the blood to pathologic levels of tissue factor

Question 7

The most common inherited factor deficiencies are the

Answer 7

hemophilias

Question 8

An isolated abnormal prothrombin time (PT) suggests

Answer 8

FVII deficiency 7

Question 9

prolonged activated partial thromboplastin time (aPTT) indicates most commonly

Answer 9

hemophilia or FXI deficiency

Question 10

The prolongation of both PT and aPTT suggests deficiency of

Answer 10

FV, FX, FII, or fibrinogen

Question 11

What is the mainstay of treatment for TTP?

Answer 11

Plasma exchange

Question 12

What is the PENTAD for TTP?

Answer 12

Microangiopathic hemolytic anemia
Thrombocytopenic purpura
Neurological abnormalities
Fever
Renal disease

Question 13

WHO considers how much % of blast to distinguish AML from MDS?

Answer 13

20% last in the marrow

Question 14

What clinical finding differentiates PCV from other causes of ERYTHROCYTOSIS?

Answer 14

Skin itching on contact with water

Aquagenic pruritus

Question 15

In most PV Patients, once an iron-deficient state is achieved , PHLEBOTOMY is usually only required at

Answer 15

3months intervals.

Question 16

Iron chelating agents for hemosiderosis

Answer 16

Deferoxamine (IV PARENTERAL)

Desferasirox (oral)

Question 17

The gold standard diagnosis of paroxysmal nocturnal hemoglobinuria (PNH)

Answer 17

Flow cytometry

Question 18

Preferred treatment for PNH

Answer 18

ECULIZUMAB

Question 19

Pure red cell a plasma is compatible with long term survival with supportive care alone such as

Answer 19

A combination of ERYTHROCYTE TRANSFUSIONS and IRON chelation.

Question 20

For persistent B19 parvovirus infection, almost all patients respond to

Answer 20

IV Immunoglobulin therapy

Question 21

The majority of patients with IDIOPATHIC PRCA respond favorably to IMMUNOSUPPRESSION such as

Answer 21

Glucocorticoids
Cyclosporine 
ATG
Azathioprine
Cyclophosphamide

Question 22

Mutation of this gene plays a central role in the pathogenesis of Polycythemia Vera as well as other myeloproliferative neoplasias

Answer 22

JAK 2

Question 23

Is a distinctive manifestation characterized by chest pain, tachypnea , fever, cough and arterial oxygen desaturation. It can mimic pneumonia , PE, bone marrow infarction and embolism, MI or in situ lung infarction.

Answer 23

Acute chest syndrome

Question 24

What drug should be considered for patients experiencing repeated episodes of acute chest syndrome or with more than 3 crises per year requiring hospitalization

Answer 24

Hydroxyurea

Question 25

is the major protease enzyme of the fibrinolytic system

Answer 25

plasmin

Question 26

The most sensitive test for DIC is the

Answer 26

FDP level | Fibrin degradation product level

Question 27

The skin and mucous membranes may be pale if thehemoglobin is

Answer 27

<80–100 g/L (8–10 g/dL)

Question 28

(; an indirect measure of serum transferrin)

Answer 28

TIBC

Question 29

Marked alterations in the red cell indices usually reflect disorders of

Answer 29

maturation or iron deficiency

Question 30

hyperviscosity and thrombosis (both venous and arterial), because the blood viscosity increases logarithmically at hematocrits >

Answer 30

55%

Question 31

True or false | At least 75% of all cases of anemiaare hypoproliferative in nature

Answer 31

True

Question 32

Suppression of EPO BY reduced tissue needs-for OXYGEN from metabolic disease such as

Answer 32

hypothyroidism

Question 33

Patients with the anemia of acute or chronic inflammation show a distinctive pattern of serum iron (),TIBC (), percent transferrin saturation (), and serumferritin ()

Answer 33

serum iron (low),TIBC (normal or low), percent transferrin saturation (low), and serumferritin (normal or high)

Question 34

These changes in iron values are brought about by_______, the iron regulatory hormone that is produced bythe liver and is increased in inflammation

Answer 34

hepcidin

Question 35

distinct pattern of results is noted in | mild to moderate iron deficiency

Answer 35

low serumiron, high TIBC, low percent transferrin saturation, low serum ferritin)

Question 36

The diagnosis of β-thalassemia major is readily made during childhood on the basis of severe anemia accompanied by the characteristic signs of massive ineffective erythropoiesis:

Answer 36

hepatosplenomegaly, profound microcytosis, a characteristic blood smear, and elevatedlevels of HbF, HbA2, or both

Question 37

folic acid should be given as a supplement before and throughout pregnancy to prevent megaloblasticanemia and reduce the incidence of NTDs, even in countries withfortification of the diet

Answer 37

400 μg daily

Question 38

In women who have had a previous fetus with an NTD,what is the recommended dose when pregnancy is contemplated and throughout the subsequent pregnancy.

Answer 38

5 mg daily (5000 mcg)

Question 39

True or false: Cobalamin should be given routinely to all patients whohave had a total gastrectomy or ileal resection

Answer 39

True

Question 40

Dosage for cobalamin therapy Replenishment of body stores should be complete with

Answer 40

six1000-μg IM injections of hydroxocobalamin given at 3- to 7-dayintervals

Question 41

Causes of Cobalamin Deficiency Sufficiently Severe toCause Megaloblastic Anemia

Answer 41

``` Pernicious anemia Congenital absence of intrinsic factor Total or partial gastrectomy Intestinal stagnant loop syndrome: jejunaldiverticulosis, ileocolic fistula, anatomic blind loop,intestinal stricture, Ileal resection and Crohn’s disease Selective malabsorption with proteinuria Tropical sprue Transcobalamin II deficiency Fish tapeworm ```

Question 42

characterized by microangiopathic HA with presence of Fragmented erythrocytes in the peripheral blood smear, thrombocytopenia (usually mild), and acute renal failure.

Answer 42

Familial (Atypical) Hemolytic-Uremic Syndrome (aHUS

Question 43

warm antibody AIHA | WARM, MOSTLY IgG, OPTIMAL-TEMPERATURE 37°C;OR MIXED

Answer 43

SLE CLL Majority: currently most common culprit drugs are cefotetan,ceftriaxone, piperacillin

Question 44

Classification of Acquired Immune Hemolytic Anemias: COLD, MOSTLY IgM, OPTIMAL-TEMPERATURE 4°C–30°C

Answer 44

EBV CMV Mycoplasma infection:paroxysmal cold hemoglobinuria Waldenstróm’s disease Lymphoma

Question 45

This triad makes PNH Paroxysmal Nocturnal Hemoglobinuria

Answer 45

i) hemolysis ii) pancytopenia iii) a distinct tendency To venous thrombosis

Question 46

The definitive diagnosis of PNH must be based on the demonstration that a substantial proportion of the patient’s red cells have an increased susceptibility to

Answer 46

complement (C)

Question 47

replacement of the bone marrow by fat is apparent in the morphology of the biopsy specimen and Magnetic resonance imaging (MRI) of the spine

Answer 47

APLASTIC ANEMIA

Question 48

Caféau lait spots and short stature suggest;

Answer 48

Fanconi anemia

Question 49

characterized by anemia, reticulocytopenia, and absent or rare erythroid precursor cells in the bone marrow + occasional THYMOMA (thrombocytopenia with amegakaryocytosis and neutropenia without marrow myeloid cells in agranulocytosis)

Answer 49

Pure red cell aplasia

Question 50

are a heterogeneous group of hematologic disorders broadlycharacterized by both (1) cytopenias due to bone marrow failure and(2) a high risk of development of AML

Answer 50

Myelodysplasia

Question 51

Childrenwith Down syndrome are susceptible to

Answer 51

MDS

Question 52

What is the only management for MDS

Answer 52

Only hematopoietic stem cell transplantation offers cure of MDS

Question 53

Which hematologic abnormality can be found in anemia of liver disease

Answer 53

Shortened red cell survival

Question 54

What Level of prothrombin time is a good indicator of the severity of clotting factor consumption?

Answer 54

>1.5 x the normal

Question 55

Which agent is the most consistently associated with aplastic anemia?

Answer 55

Hydantoins

Question 56

What type of diarrheal infection is associated with HUS

Answer 56

E. coli