RBC Disorders Flashcards

1
Q

What is hemoglobin

A

Conjugated protein that serve as vehicle for transportation of o2 and co2

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2
Q

Packed red cell volume
Raised blood cells of RBC to the total blood called

A

Hematocrit

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3
Q

Anemia characteristics and features

A

Reduce in total circulating RBC
May be MCV IS micro, normo, macrocytic
MCHc is hypo or normochromic

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4
Q

Microcytic anemia disease

A

T-Thalaseemia
A-Anemia of chronic disease
I- Iron deficiency
L-Lesd poisoning

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5
Q

Macrocytic disease

A

Megaloblastic and non megaloblastic
Defective in DNA synthesis, they are
Folate deficiency
Vit B12 deficiency
Orotic aciduria
Fanconi anemia

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6
Q

Hemolytic anemia common features

A

Shortened life span
Elevated erythropoietin
Reticulocytosis
Accumulation of hemoglobin degraded products like jaundice, gallstones and hemosiderosis

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7
Q

Hereditary sperocytosis

A

It’s a dominant disorder.
Defect in cell membranes skeleton like spectrin, ankyrin, band 4.2,and band 3

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8
Q

Features of hereditary sperocytosis

A

Normocytic
Hyperchromic
Intrinsic damage and extravascular hemolysis in spleen
Anisocytosis
Spherocytes
Howen jolly bodies

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9
Q

Glucose 6 phosphate dehydrogenase deficiency

A

X linked recessive

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10
Q

Causes of hemolysis in G6PD

A

Intrinsic and episodic hemolysis
Both intra and extravascular hemolysis
In intra heinz bodies cause membrane damage
Extravascular has heinz bodies are bittenoff by spleen

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11
Q

Main features seen in G6PD deficiency

A

Heinz bodies
Bite cells or degmacytes
Protective against p. Falciparum

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12
Q

Sickle cell disease

A

Autosomal recessive
Point mutation on 6th codon of beta globin chain glutamine to valine
Extramedullary hematopoisis is seen

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13
Q

Sickle cell disease features

A

Sickle cells called drepanicytes
Target cells called codocytes
Howen jolly bodies
None marrow expansion
Anisocytosis and poikilocytosis
Vasoocculisive crisis

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14
Q

Alpha -Thalaseemia results in mutation in which chromosome

A

Ch 16 of 4 genes

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15
Q

Beta thalaseemia mutation in

A

Ch 11 of 2 genes

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16
Q

Thalaseemia main featurrs

A

Extravascular hemolysis
Anisocytosis
Microcytic, hypochromic
Poikilocytosis
Target cells
Beta thalaseemia major called as cooley anemia which has crewcut appearance on x ray

17
Q

Paraxysomal nocturnal hemoglobinuria identification

A

It’s immunohemolytic anemia
It’s identifird by negative expression of CD 55 and 59
Mutation in phosphotidylinositol glycan complementation group A gene

18
Q

PNH which type of hemolysis

A

Both intra and extravascular by complement fixation and spleen sequestration

19
Q

Large bizzare multinuckeated marcroohages seen in

A

Megaloblastic anemia

20
Q

Features of megaloblastic anemia

A

Macrocytic, normochromic
Anisocytosis, poikilocytosis
Low reticulocyte count
Hypersegmented neutrophils

21
Q

Pencil cells and plummer vinson syndrome triad is seen in

A

Iron deficiency anemia

22
Q

Microcutic, hypochromic anemia

A

Iron deficiency anemia

23
Q

Serum ferritin is high andblow in which disorders

A

Anemia of chronic inflammation and iron deficiency anemia respectively

24
Q

Aplastic anemia

A

Chronic primary hematopoietic failure and pancytopenia

25
Q

Reticulocytopenia is the rule in in which disease

A

Aplastic anemia

26
Q

Tear drop cells and leukoerythroblastosis is seen in

A

Myelophthisic anemia

27
Q

Relative polycythemia of which etiology has vomiting stress etc.. Called as

A

Stress polycythemia and gaisbock syndrome

28
Q

Prolonged prothrombin time (PT) results in

A

Deficiency or dysfunction in factor v, vii, x prothrombin and fibrinogen
Measures extrinsic and common pathway of coagulation

29
Q

Prolonged Partial thromboplastin time (Ptt) results in

A

Deficiency of intrinsic factors like factor v, viii, ix, x, xi, xii, prothrombin and fibrinogen

30
Q

High platelet count seen in

A

Myeloproliferative neoplasm

31
Q

Autoimmune mediated destruction of platelets seen in

A

Chronic immune thrombocytopenic purpura

32
Q

Parvovirus B19 leads to

A

Aplastic anemia

33
Q

Haemophilia A patients have

A

Factor 8 deficiency and prolonged ptt and normal pt

34
Q

Haemophilia B

A

Defect in factor 9 or Christmas factor

35
Q

Excessive activation of coagulation and formation of thrombi in microvasculature

A

Disseminated intravascular coagulation