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11 Cards in this Set
- Front
- Back
- 3rd side (hint)
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What is the difference between pancytopenia & aplastic anaemia? |
Pancytopenia is reduction of all three cell lines. Aplastic anaemia is Pancytopenia due to bone marrow hypoplasia. |
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Causes for Pancytopenia |
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Causes for Aplastic anaemia |
Primary- congenital fanconi & non-fanconi types Idiopathic acquired Secondary- Ionising radiation Chemicals Drugs Viruses ( Non-A, Non-B, non-C, non-G hepatitis, EBV ) Autoimmune disease eg-SLE Transfusion associated GVHD Thymoma ( more in red cell aplasia ) |
Primary & secondary |
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Pathogenesis of Aplastic anaemia |
Reduction of haemopoietic pluripotential stem cells With Fault in remaining stem cells Or Immune process against remaining stem cells, Making them unable to proliferate and differentiate.. |
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Congenital - Fanconi type inheritance |
Autosomal recessive 16 genes involved |
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Fanconi anaemia features |
Growth retardation Skeletal defects ( absent radii/ thumbs, microcephaly ) Renal defects (pelvic / horse shoe kidney ) Skin ( areas of hyper / hypopigmentation ) Learning disability |
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What is the most common type of Aplastic anaemia? |
Idiopathic acquired Aplastic anaemia 2/3 of all aplastic |
Thought to be autoimmune |
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Criteria for aplastic anaemia |
1. Hg less than 10g/dl Macrocytic or normocytic normochromic 2.Neutrophils less than 1.5 x 10 9 3.Platelet count less than 50 x 10 9 At least 2 of above with, 4.no abnormal blood cells in peripheral blood film 5.marrow hypoplasia with around 75% fat infiltrate |
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What is paroxysmal nocturnal haemoglobinuria |
* acquired x chromosome gene mutation * defects in phospatidylinositol glycan protein class A (PIG-A) *PIG-A necessary to make GPI anchor *GPI linked proteins absent in all cells from mutated stem cells Eg- CD 55 DAF decay activating factor CD 59 MIRL membrane inhibitor of reactive lysis * If above proteins absent, RBC are sensitive to lysis by complement *chronic intravascular haemolysis |
Pathogenesis |
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What is the clinical triad in Paroxysmal nocturnal haemoglobinuria? |
*Chronic intravascular haemolysis *Venous thrombosis (recurrent thrombosis of large vessels eg- hepatic, mesenteric causing ab.pain , hepatic) *Bone marrow failure Other features *Haemosiderinuria -may worsen anaemia due to ion defficiency *haptoglobulins absent *free Hb -damage kidney -remove NO from smooth muscles causing dysphagia & pulmonary hypertension *arterial thrombosis eg-MI, CVA |
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What is the classification of pure red cell aplasia? |
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Acute, transient Chronic congenital Chronic acquired |
