RBC and Platelet Disorders Flashcards
hallmarks of all hemolytic anemias
Erythroid hyperplasia, retiulocytosis,
If sever then etramedullary hematopoeisis
Hallmarks of extracorpuscular hemolytic anemia
Hyperbilirubinemia, jaundice, pigement stones
Hallmarks of intravascular hemolysis
Hemoglobinemia, hemoglobinuria, hemosiderosis
pausible IDA(not a hallmark)
Common feature of extra and intra vascular hemolytic anemia
Decreased level of haptoglobin
Name the 3 etiologies of extracorpuscular hemolytic anemia
- Antibody mediated
- Malarial infection
- Mechanical trauma like DIC & TTP
Gene defect in hereditary spherocytosis
Ankyrin band 3 or spectrin
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
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
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
Target cells showing dark red puddle in center seen in?
B thalessemia minor and alpha thalessemia trait
Marked microcytosis, hypochromia, poikilocytosis, anisocytosis seen in ?
B thalessemia major
Hb bart and hbH seen in
B4 and gamma4 tetramers in alpha thalessemia
Name 5 systemic features of beta thalessemia major
- Skeletal deformities
- Hepatomegaly
- Splenomegaly
- Lymphadenopathy
- Hemosiderosis
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
Genes involved in alpha and beta chain defects in thalassemia
Chr 11 alpha
Chr 16 beta
What are heinz bodies ?
Denatured oxidised hemoglobin seen in G6PD Deficiency intravasular hemolytic anemia
G6PD is caused due to gene defect on which chromosome?
X chromosome
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
Name 6 drugs causing oxidative stress and transient intravascular hemolysis in g6pd deficiency
- Aspirin
- Sulfonamide
- Nitrofurantoin
- Vit K derivatives
- Primaquine
- Phenacetin
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
Frank erythrocytosis, nibbles antibody coated rbc, spherocytes seen in
Warm ab type hemolytic anemia
Which Ig is involved in warm ab type hemolytic anemia
IgG
IgA rarely
Secondary caused of warm ab type anemia
CLL, SLE
drugs - alpha methyl dopa, penicillin, quinidine
Cold ab type anemia antibody and temp involved and complement coated on rbc
Ig M below 30 degree C
, c3 coated in cold weather
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