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Pathology > RBC and Platelet Disorders > Flashcards

RBC and Platelet Disorders Flashcards

1
Q

hallmarks of all hemolytic anemias

A

Erythroid hyperplasia, retiulocytosis,
If sever then etramedullary hematopoeisis

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2
Q

Hallmarks of extracorpuscular hemolytic anemia

A

Hyperbilirubinemia, jaundice, pigement stones

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3
Q

Hallmarks of intravascular hemolysis

A

Hemoglobinemia, hemoglobinuria, hemosiderosis
pausible IDA(not a hallmark)

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4
Q

Common feature of extra and intra vascular hemolytic anemia

A

Decreased level of haptoglobin

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5
Q

Name the 3 etiologies of extracorpuscular hemolytic anemia

A
  1. Antibody mediated
  2. Malarial infection
  3. Mechanical trauma like DIC & TTP
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6
Q

Gene defect in hereditary spherocytosis

A

Ankyrin band 3 or spectrin

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7
Q

Consequence of gene defect in HS

A

Weaken the vertical interactions between membrane skeleton and intrinsic RBC membrane proteins which leads to spherical shape of cells and sequestration in spleen

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8
Q

Morphology seen in hereditary spherocytosis

A

Enlargement of spleenic cords and splenomegaly 500 to 1000g
Dark red with no central pallor spherocytes
Extramedullary hematopoeisis and reticulocytosis
Cholelithiasis in some

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9
Q

A 13 year old girl presents with pallor, icterus, fatigue since 10 days and shows splenomegaly O/E. Microscopy of peripheral smear shows dark red cells lacking central pallor along with reticulocytes and bone marrow biopsy shows bone marrow hyperplasia. Whats your diagnosis and management?

A

Hereditary spherocytosis.
Confirm gene defect ankyrin band 3 or spectrin in RBC by family history and lab diagnosis
Spleenectomy

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10
Q

Target cells showing dark red puddle in center seen in?

A

B thalessemia minor and alpha thalessemia trait

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11
Q

Marked microcytosis, hypochromia, poikilocytosis, anisocytosis seen in ?

A

B thalessemia major

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12
Q

Hb bart and hbH seen in

A

B4 and gamma4 tetramers in alpha thalessemia

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13
Q

Name 5 systemic features of beta thalessemia major

A
  1. Skeletal deformities
  2. Hepatomegaly
  3. Splenomegaly
  4. Lymphadenopathy
  5. Hemosiderosis
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14
Q

A 22 year old suffered from growth retardation as an infant and now has cardiac dysfunction from secondary hemachromatosis. Treated with iron chelators and blood tranfusions done, she is scheduled for HSC stem cell transplant. Make your diagnosis.

A

Beta thalessemia major

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15
Q

Genes involved in alpha and beta chain defects in thalassemia

A

Chr 11 alpha
Chr 16 beta

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16
Q

What are heinz bodies ?

A

Denatured oxidised hemoglobin seen in G6PD Deficiency intravasular hemolytic anemia

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17
Q

G6PD is caused due to gene defect on which chromosome?

A

X chromosome

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18
Q

What are bite cells, where are they seen and how are they made?

A

Seen in g6pd deficiency anemia. These are damaged RBCs due to oxidative stress. Bites are created by phagocytes in attempt to pluck out the heinz bodies

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19
Q

Name 6 drugs causing oxidative stress and transient intravascular hemolysis in g6pd deficiency

A
  1. Aspirin
  2. Sulfonamide
  3. Nitrofurantoin
  4. Vit K derivatives
  5. Primaquine
  6. Phenacetin
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20
Q

What is phosphatidylinositol( PIG) glycan? What gene governs it? What is its function? What does its defect cause?

A

Membrane anchor for many rbc membrane proteins.
PIGA gene
Paroxysmal noctural hemoglobinuria

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21
Q

Frank erythrocytosis, nibbles antibody coated rbc, spherocytes seen in

A

Warm ab type hemolytic anemia

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22
Q

Which Ig is involved in warm ab type hemolytic anemia

A

IgG
IgA rarely

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23
Q

Secondary caused of warm ab type anemia

A

CLL, SLE
drugs - alpha methyl dopa, penicillin, quinidine

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24
Q

Cold ab type anemia antibody and temp involved and complement coated on rbc

A

Ig M below 30 degree C
, c3 coated in cold weather

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25
Q

Raynaud phenomenon seen in which hemolytic anemia

A

Cold ab type

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26
Q

Acute and chronic causes of IgM type hemolytic anemia

A

Acute - mycoplasma, infectious mononucleosis
Chronic - lymphoplasmacytic lymphoma

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27
Q

Blender effect seen in which anemia

A

Extravascular hemolytic anemia due to mechanical trauma

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28
Q

What is blackwater fever?

A

Malaria complication
Massive intravascular hemolysis
Hemoglobinemia, hemoglobinuria
Jaundice

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29
Q

Morphology seen in malaria?

A

Hemolytic anemia
hematin discolouration in organs
Marked hyperplasia of mononuclear phagocytes
MASSIVE splenomegaly

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30
Q

Sources of iron in diet

A

Meat, poultry, vegetables

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31
Q

Mention cofactors, transporters and enzymes involved in iron metabolism

A

Ferrous reductase, DMT1, ferroportin, ceruloplasmin, hephaestin, transferrin, ferritin

32
Q

Mention four causes of IDA

A
  1. Celiac disease malabsorption
  2. Chronic blood loss
  3. Vegetarian diet
  4. Helminthic infestation
33
Q

An adolescent girl came with complaints of weakness, pallor and O/E shows spooning of nails. Her mother also tells an unusual habit of hers to consume non foodstuff like dirt or clay(pica). Diagnosis?

A

Iron deficiency anemia

34
Q

Name neoplasms causing anemia

A

Hodgkin lymphoma
Lung and breast neoplasms

35
Q

Name three infectious disorders causing anemia

A

Endocarditis
Osteomyelitis
Lung abscess

36
Q

2 immune disorders causing anemia

A

Rheumatoid arthritis
Regional enteritis

37
Q

Pathogenic cause for anemia in chronic inflammation

A

Hepcidin released d/t IL6

38
Q

Profile of patient blood
MCV 136fL
Hypersegmented neutrophils
Giant metamyelocytes
Megaloblasts
Clumping of chromatin

A

Megaloblastic anemia

39
Q

True or false
DNA abnormalities seen in megaloblastic anemias are due to issues with thymine synthesis due to B12 or folate deficiency

40
Q

Name three conditions where folate deficiency is possible due to increased demand of folate

A
  1. Pregnancy
  2. Sickle cell disease
  3. Chronic hemolytic anemia
41
Q

Name 3 situations where folate absorption is interfered with

A

Drugs like phenytoin and methotrexate
Tropical sprue
Celiac disease

42
Q

Differentiating points between folate and B12 deficiency anemia

A
  1. Red cell and serum folate levels
  2. Neurological deficits in B12 only
  3. Sore tongue seen in folate deficiency
43
Q

What is the most common cause of B12 deficiency anemia ?

A

Pernicious anemia
Caused by autoimmune attack on parietal cells preventing absorption of B12 due to lack of intrinsic factor as seen in chronic atrophic gastritis

44
Q

Mention 7 causes of B12 deficiency anemia apart from pernicious anemia

A

Crohns disease
Tropical sprue
Whipple’s disease
Achlorhydria
Gastric atrophy
Ileal resection
Gastrectomy

45
Q

Name the main neurological lesion associated with b12 deficiency anemia

A

Demyelination of posterior and lateral columns of spinal cord

46
Q

A 23 year old woman came with easy fatiguability, pallor, mild icterus, beefy red tongue, parasthesia. What is your provisional diagnosis?

A

B12 deficiency anemia

47
Q

What are two serious complications of b12 deficiency anemia

A

CHF
gastric carcinoma if pernicious anemia is present

48
Q

Supportive findings in b12 def anemia

A

Low b12 serum levels
Normal folate levels
Dramatic reticulocyte response 2-3 days on b12 administration
Hypersegmented granulocytes
Macrocytic anemia

49
Q

Mediators in aplastic anemia

A

Gamma IFN
TNF
Th1 cells

50
Q

Patient comes with weakness, pallor, breathlessness with petechiae and ecchymoses. Bond marrow biopsy shows pancytopenia. O/E no splenomegaly seen. Patient has h/o chemotherapy. Diagnosis?

A

Aplastic anemia

51
Q

Teardrop cells and leukoerythroblastosis seen in which type of anemia?

A

Myelophthistic anemia

52
Q

Relative polycythemia caused by

A

Dehydration, diuretics, loss of electrolytes by vomit or diarrhoea

53
Q

Causes of absolute polycythemia

A

Primary due to polycythemia vera
Secondary due to increased erythropoeitin production

54
Q

Mention the investigations for suspected coagulopathies

A

Prothrombin time
Partial thromboplastin time

55
Q

Test for extrinsic pathway and common pathway of coagulation and may indicate deficiency of factors 5,7,10,prothrombin, fibrinogen

56
Q

Test for intrinsic and common pathway of coagulation and shows factor deficiency of 5,8,9,10,11,12

57
Q

Normal platelet count

A

150000-450000/ul

58
Q

What are the clinical signs of inadequate platelet function?

A

Menorrhagia, excessive bleeding from bruises, nosebleeds

59
Q

What is another name for consumptive coagulopathy?

A

Disseminated intravascular coagulation

60
Q

Predisposing conditions to dic name 6

A

Obstetric complications
Adenocarcinoma
Acute promyelocytic leukemia
Sepsis due to infections
Heat stroke or burns
SLE

61
Q

2 consequences of dic

A

Fibrin thrombi
Microangiopathic hemolytic anemia

62
Q

2 syndromes caused by dic

A

Waterhouse friderichson syndrome
Sheehan postpartum pituitary necrosis

63
Q

Patient is undergoing shock,renal failure, dyspnoea, convulsions is cyanotic and comatose. Diagnosis?

A

DIC secondary to sepsis,cancer,burns,heatstroke, or sle

64
Q

A 29 y/o female is undergoing prolonged copious postpartum accompanied by petechia and ecchymoses. Diagnosis?

A

DIC secondary to obstetric complication

65
Q

Pentad of thrombocytic microangiopathies

A

Fever
Thrombocytopenia
Microangiopathic hemolytic anemia
Transient neurological deficits
Acute renal failure

66
Q

2 diseases which make up thrombocytic microangiopathies

A

HUS hemolytic uremic syndrome
TTP thrombotic thrombocytopenic purpura

67
Q

What is the damage in immune thrombocytopenic purpura

A

IgG antibodies against
IIb/IIIa Or Ib/IX complexes

68
Q

Salient features of von willebrand disease

A

Aut dom
Spontaneous bleeding of mucus membranes, menorrhagia, excessive bleeding from wounds
Type1, 2

69
Q

Most common hereditary cause of serious bleeding

A

Hemophilia A by factor 8 reduced activity

70
Q

Salient features of hemophilia A

A

X linked
Factor 8 deficiency

Hemarthroses, spontaneous bleeding
Prolonged PTT

71
Q

Christmas factor deficient in?

A

Hemophilia B factor 9

72
Q

A 20 year old male with myasthenia gravis presents with cough, dyspnoea, superior vena cava syndrome. Diagnosis?

73
Q

Classify splenomegaly

A

Massive more than 1000g
Moderate 500-1000g
Mild less than 500g

74
Q

7 causes of massive splenomegaly

A

Malaria
Lymphomas
CML
CLL
Primary meylofibrosis
Hairy cell leukemia
Gaucher disease

75
Q

3 noteworthy causes of moderate splenomegaly

A

Acute leukemias
Extravascular hemolysis immune reactions and infections causing it
Chronic congestive splenomegaly

76
Q

6 causes of mild splenomegaly

A

Acute splenitis
Acute splenic congestion
Infectious mononucleosis
SLE
Septicemia
Intra abdominal infections

77
Q

3 features of hypersplenism

A

Anemia, leukopenia, thrombocytopenia

Pathology (12 decks)

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