RBC and Platelet Disorders Flashcards by Kosha Bhuskute

hallmarks of all hemolytic anemias

Erythroid hyperplasia, retiulocytosis,
If sever then etramedullary hematopoeisis

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Hallmarks of extracorpuscular hemolytic anemia

Hyperbilirubinemia, jaundice, pigement stones

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Hallmarks of intravascular hemolysis

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

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Common feature of extra and intra vascular hemolytic anemia

Decreased level of haptoglobin

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Name the 3 etiologies of extracorpuscular hemolytic anemia

Antibody mediated
Malarial infection
Mechanical trauma like DIC & TTP

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Gene defect in hereditary spherocytosis

Ankyrin band 3 or spectrin

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Consequence of gene defect in HS

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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Morphology seen in hereditary spherocytosis

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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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?

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

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Target cells showing dark red puddle in center seen in?

B thalessemia minor and alpha thalessemia trait

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Marked microcytosis, hypochromia, poikilocytosis, anisocytosis seen in ?

B thalessemia major

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Hb bart and hbH seen in

B4 and gamma4 tetramers in alpha thalessemia

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Name 5 systemic features of beta thalessemia major

Skeletal deformities
Hepatomegaly
Splenomegaly
Lymphadenopathy
Hemosiderosis

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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.

Beta thalessemia major

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Genes involved in alpha and beta chain defects in thalassemia

Chr 11 alpha
Chr 16 beta

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What are heinz bodies ?

Denatured oxidised hemoglobin seen in G6PD Deficiency intravasular hemolytic anemia

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G6PD is caused due to gene defect on which chromosome?

X chromosome

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

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

Aspirin
Sulfonamide
Nitrofurantoin
Vit K derivatives
Primaquine
Phenacetin

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

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

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Frank erythrocytosis, nibbles antibody coated rbc, spherocytes seen in

Warm ab type hemolytic anemia

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Which Ig is involved in warm ab type hemolytic anemia

IgG
IgA rarely

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Secondary caused of warm ab type anemia

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

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

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

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Raynaud phenomenon seen in which hemolytic anemia

Cold ab type

Acute and chronic causes of IgM type hemolytic anemia

Acute - mycoplasma, infectious mononucleosis Chronic - lymphoplasmacytic lymphoma

Blender effect seen in which anemia

Extravascular hemolytic anemia due to mechanical trauma

What is blackwater fever?

Malaria complication Massive intravascular hemolysis Hemoglobinemia, hemoglobinuria Jaundice

Morphology seen in malaria?

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

Sources of iron in diet

Meat, poultry, vegetables

Mention cofactors, transporters and enzymes involved in iron metabolism

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

Mention four causes of IDA

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

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?

Iron deficiency anemia

Name neoplasms causing anemia

Hodgkin lymphoma Lung and breast neoplasms

Name three infectious disorders causing anemia

Endocarditis Osteomyelitis Lung abscess

2 immune disorders causing anemia

Rheumatoid arthritis Regional enteritis

Pathogenic cause for anemia in chronic inflammation

Hepcidin released d/t IL6

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

Megaloblastic anemia

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

True

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

1. Pregnancy 2. Sickle cell disease 3. Chronic hemolytic anemia

Name 3 situations where folate absorption is interfered with

Drugs like phenytoin and methotrexate Tropical sprue Celiac disease

Differentiating points between folate and B12 deficiency anemia

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

What is the most common cause of B12 deficiency anemia ?

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

Mention 7 causes of B12 deficiency anemia apart from pernicious anemia

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

Name the main neurological lesion associated with b12 deficiency anemia

Demyelination of posterior and lateral columns of spinal cord

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

B12 deficiency anemia

What are two serious complications of b12 deficiency anemia

CHF gastric carcinoma if pernicious anemia is present

Supportive findings in b12 def anemia

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

Mediators in aplastic anemia

Gamma IFN TNF Th1 cells

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?

Aplastic anemia

Teardrop cells and leukoerythroblastosis seen in which type of anemia?

Myelophthistic anemia

Relative polycythemia caused by

Dehydration, diuretics, loss of electrolytes by vomit or diarrhoea

Causes of absolute polycythemia

Primary due to polycythemia vera Secondary due to increased erythropoeitin production

Mention the investigations for suspected coagulopathies

Prothrombin time Partial thromboplastin time

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

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

PTT

Normal platelet count

150000-450000/ul

What are the clinical signs of inadequate platelet function?

Menorrhagia, excessive bleeding from bruises, nosebleeds

What is another name for consumptive coagulopathy?

Disseminated intravascular coagulation

Predisposing conditions to dic name 6

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

2 consequences of dic

Fibrin thrombi Microangiopathic hemolytic anemia

2 syndromes caused by dic

Waterhouse friderichson syndrome Sheehan postpartum pituitary necrosis

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

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

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

DIC secondary to obstetric complication

Pentad of thrombocytic microangiopathies

Fever Thrombocytopenia Microangiopathic hemolytic anemia Transient neurological deficits Acute renal failure

2 diseases which make up thrombocytic microangiopathies

HUS hemolytic uremic syndrome TTP thrombotic thrombocytopenic purpura

What is the damage in immune thrombocytopenic purpura

IgG antibodies against IIb/IIIa Or Ib/IX complexes

Salient features of von willebrand disease

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

Most common hereditary cause of serious bleeding

Hemophilia A by factor 8 reduced activity

Salient features of hemophilia A

X linked Factor 8 deficiency Hemarthroses, spontaneous bleeding Prolonged PTT

Christmas factor deficient in?

Hemophilia B factor 9

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

Thymoma

Classify splenomegaly

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

7 causes of massive splenomegaly

Malaria Lymphomas CML CLL Primary meylofibrosis Hairy cell leukemia Gaucher disease

3 noteworthy causes of moderate splenomegaly

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

6 causes of mild splenomegaly

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

3 features of hypersplenism

Anemia, leukopenia, thrombocytopenia

RBC and Platelet Disorders Flashcards

(77 cards)