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33 Cards in this Set
- Front
- Back
Pinpoint, non blanching hemorrhages as in the picture are called |
Petechiae |
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Petechiae denotes what? |
Decreased platelet number |
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Pseudothrombocytopenia |
Due to hypocalcemia because of ETDA in CBC tubes. Mechanism: Platelet agglutination via antibodies |
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Most common cause of Thrombocytopenia |
Non prescription drugs and herbals. |
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Most common non-iatrogenic causes of Thrombocytopenia |
Infections (bacterial and viral) - decreases production and survival. |
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Examples of immuneediated thrombocytopenia |
Infectious mononucleosis. Early HIV (In Late HIV - decreased production). Infection associtaed ITP in children. |
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What is it? What are the DDs? |
Macro-thrombocytopenia DIfferentials : May hegglin anomaly Sebastian syndrome Epstein's syndrome Fechter syndrome |
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Evan's syndrome |
AIHA + ITP |
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Examples of X linked inherited thrombocytopenia syndromes X linked Thrombocytopenia |
Wiskott Aldrich syndrome Dyshematopoietic syndrome from GATA1 mutation WAS GATA |
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A R thrombocytopenias |
CAT (congenital amegakaryocytic thrombocytopenias) Thrombocytopenia with absent radii Bernard Soulier syndrome A B CAT |
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Mortality rate in TTP without plasma exchange? |
85-100% without treatment to 10-30% with plasma exchange |
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What is Upshaw Schulman syndrome? |
Inherited TTP |
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Pathogenesis of idiopathic TTP |
Antibodies to ADAMTS13 (Enzyme level < 10% are clearly associated) |
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People predisposed to TTP |
Pregnancy. HIV |
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Drug induced TTP |
Antibody formation: Toclopidine, Clopidogrel. Endothelial injury: Cyclosporine Mitomycin C CNI Quinine (Decreasing doses in the second group decreases microangiopathy) |
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True or False. Idiopathic TTP is more common in men. |
False. Idiopathic TTP is related to antibodies to ADAMTS13. It is more common with women. |
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In a patient presenting with new thrombocytopenia (with or without other features of TTP), how will you evaluate? |
Lab data to ruleout DIC and evaluate for MAHA. Findings in favour of TTP diagnosis: Increased LDH Indirect bilirubin Low haptoglobulin High Retic count With Coombs negativity. Peripheral smear for schistiocytes. |
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True or false. Immature RBCs in peripheral blood in TTP is due to hemolysis and infarction of small vasculature in marrow. |
True |
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Duration of plasma exchange in TTP |
Until platelet count normalising or signs of hemolysis resolving for 2 days |
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TRUE OR FALSE. Gluco corticoids can be used in TTP. |
True. |
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Other agents that can be used in TTP |
Rituximab Vincristine Cyclophosphamide Splenectomy |
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Relapse rate in TTP |
25-50% early relapse in 30 days. Relapse correlates with severity of ADAMTS13. |
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Need for dialysis in children with typical HUS |
40%, atleast for some period. |
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Overall mortality in typical HUS |
< 5 % |
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Overall mortality in patients with aHUS |
25% |
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True or flase. aHUS is a diagnosis of exclusion. |
True |
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Treatment for aHUS. |
Eculizumab |
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Reason behind HUS. |
Shiga like toxin form EHEC O157:H7. |
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Reason behind aHUS. |
Chronic complement activation. |
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Reason behind TTP. |
ADAMTS 13 deficiency |
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True or false.
Plasma exchange is useful in aHUS and so plasma exchange is done.
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False. aHUS may be initially treated with plasma exchange until ADAMTS13 levels are obtained and the diagnosis more clear. (ie TTP rules out)
Plasma exchange has no benefit in clinical outcomes. |
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DIfferentials for Thrombocytosis |
Iron deficiency. Reactive Thrombocytosis (inflammation, cancer or infection). MPNs. Rarely, 5q deletion MDS |
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Enzymes inportant for metabolism and regeneration of vitamin K |
ɣ glutamyl carboxylase (GGCX) Epoxide reductase (VKORC1) |
