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HARRISSONS > Hematology > Flashcards

Hematology Flashcards

1
Q

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

A

methylcobalamin,

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2
Q

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

A

TC I

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3
Q

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

A

0.5 to 1gram of IRON

sustained tx 6-12months needed to achieve this

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4
Q

True or false:

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

A

True

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5
Q

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

A

Purpura fulminans

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6
Q

The central mechanism of DIC is the

A

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

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7
Q

The most common inherited factor deficiencies are the

A

hemophilias

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8
Q

An isolated abnormal prothrombin time (PT) suggests

A

FVII deficiency 7

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9
Q

prolonged activated partial thromboplastin time (aPTT) indicates most commonly

A

hemophilia or FXI deficiency

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10
Q

The prolongation of both PT and aPTT suggests deficiency of

A

FV, FX, FII, or fibrinogen

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11
Q

What is the mainstay of treatment for TTP?

A

Plasma exchange

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12
Q

What is the PENTAD for TTP?

A 
Microangiopathic hemolytic anemia
Thrombocytopenic purpura
Neurological abnormalities
Fever
Renal disease
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13
Q

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

A

20% last in the marrow

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14
Q

What clinical finding differentiates PCV from other causes of ERYTHROCYTOSIS?

A

Skin itching on contact with water

Aquagenic pruritus

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15
Q

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

A

3months intervals.

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16
Q

Iron chelating agents for hemosiderosis

A

Deferoxamine (IV PARENTERAL)

Desferasirox (oral)

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17
Q

The gold standard diagnosis of paroxysmal nocturnal hemoglobinuria (PNH)

A

Flow cytometry

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18
Q

Preferred treatment for PNH

A

ECULIZUMAB

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19
Q

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

A

A combination of ERYTHROCYTE TRANSFUSIONS and IRON chelation.

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20
Q

For persistent B19 parvovirus infection, almost all patients respond to

A

IV Immunoglobulin therapy

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21
Q

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

A 
Glucocorticoids
Cyclosporine 
ATG
Azathioprine
Cyclophosphamide
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22
Q

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

A

JAK 2

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23
Q

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.

A

Acute chest syndrome

24
Q

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

A

Hydroxyurea

25
Q

is the major protease enzyme of the fibrinolytic system

A

plasmin

26
Q

The most sensitive test for DIC is the

A

FDP level

Fibrin degradation product level

27
Q

The skin and mucous membranes may be pale if thehemoglobin is

A

<80–100 g/L (8–10 g/dL)

28
Q

(; an indirect measure of serum transferrin)

A

TIBC

29
Q

Marked alterations in the red cell indices usually reflect disorders of

A

maturation or iron deficiency

30
Q

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

A

55%

31
Q

True or false

At least 75% of all cases of anemiaare hypoproliferative in nature

A

True

32
Q

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

A

hypothyroidism

33
Q

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

A

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

34
Q

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

A

hepcidin

35
Q

distinct pattern of results is noted in

mild to moderate iron deficiency

A

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

36
Q

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:

A

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

37
Q

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

A

400 μg daily

38
Q

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.

A

5 mg daily (5000 mcg)

39
Q

True or false:

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

A

True

40
Q

Dosage for cobalamin therapy

Replenishment of body stores should be complete with

A

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

41
Q

Causes of Cobalamin Deficiency Sufficiently Severe toCause Megaloblastic Anemia

A 
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
42
Q

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

A

Familial (Atypical) Hemolytic-Uremic Syndrome (aHUS

43
Q

warm antibody AIHA

WARM, MOSTLY IgG, OPTIMAL-TEMPERATURE 37°C;OR MIXED

A

SLE
CLL

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

44
Q

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

A

EBV
CMV

Mycoplasma infection:paroxysmal cold hemoglobinuria

Waldenstróm’s disease Lymphoma

45
Q

This triad makes PNH Paroxysmal Nocturnal Hemoglobinuria

A

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

46
Q

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

A

complement (C)

47
Q

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

A

APLASTIC ANEMIA

48
Q

Caféau lait spots and short stature suggest;

A

Fanconi anemia

49
Q

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)

A

Pure red cell aplasia

50
Q

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

A

Myelodysplasia

51
Q

Childrenwith Down syndrome are susceptible to

A

MDS

52
Q

What is the only management for MDS

A

Only hematopoietic stem cell transplantation offers cure of MDS

53
Q

Which hematologic abnormality can be found in anemia of liver disease

A

Shortened red cell survival

54
Q

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

A

> 1.5 x the normal

55
Q

Which agent is the most consistently associated with aplastic anemia?

A

Hydantoins

56
Q

What type of diarrheal infection is associated with HUS

A

E. coli

HARRISSONS (62 decks)

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