Hematology
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| Examples of platelet disease | Acute Thrombocytopenia; HIT; ITP; TTP; HUS
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| Causes of thrombocytopenia (decrease in plt numbers) | Decreased prodn (BM prob); inc destn (meds, immune mediated, DIC); Sequestration (Splenomegaly)
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| ITP diagnosis is: | one of exclusion (ie, notable for what is not present)
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| ITP findings | Neg FH/SH/PMH. Labs = acute thrombocytopenia; all else normal. Bruising, petechiae, gingival bleeding, hemorrhagic bullae in kids
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| ITP tx | Immunosuppression (prednisone 1mg/kg/day; rituximab; cyclophosphamide ). Immune modulation (IVIg, splenectomy). Stimulate platelet production (eltrombopag, romiplostin)
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| 2 types of HIT | 1: transient decrease. 2: immune mediated (significant)
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| HIT mechanism | Heparin combines w/PF4; this plus Abs -> platelet activation -> limb or life threatening thrombosis
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| HIT PMHx | On heparin 4-10 days; Inflammation/necrosis?; limb asymmetry? ; Cool/pulseless extremities?
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| HIT tx | Stop all heparin products (inc hep lines & flushes & LMWHs); prompt tx w/alt. anti-coagulation: Direct Thrombin Inhibitor (Argatroban, Lepirudin, Bivalirudin); Fondaparinux (synthetic anti-coagulant); warfarin after plt normal; w/alt anticoag; 6-12 wks
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| Increase in ultra-high molecular weight multimers of von Willebrand factor (2/2 deficiency vWF proteolytic enzyme) = | TTP
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| Effects of TTP | Intra-vascular platelet aggregation; systemic manifestations (CNS, Renal, Cardiac, Hematologic)
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| H&P for TTP | mental status changes; Sx/Sx related to end-organ damage and those of acute & idiopathic thrombocytopenia
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| TTP tx | Emergent large-volume plasma plasmapheresis; RBCs and fresh plasma returned to patient. Possible prednisone & antiplatelet tx
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| Platelet release involves: | Release of PF3 (Platelet factor 3); intracellular Ca (enhances further activation); thromboxane A2
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| Adult ITP is usually: | chronic (as opposed to children, no viral infxn).
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| Most common drug causing ITP | Heparin
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| TTP clinical findings | Pentad: MAHA; thrombocytopenia; CNS abnormalities; fever; renal dysfunction
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| HUS: mental status changes? | No
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| HUS findings | Thrombocytopenia
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| Dx similar to TTP but without neuro symptoms | HUS
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| AutoAb to ADAMTS13 seen in: | TTP
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| HUS prognosis | Self-limiting in kids. 40% mortality in adults; CKD in 80%
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| Platelet aggregation abnormal with ristocetin, which corrects with the addition of normal platelets, in: | Bernard-Soulier Syndrome
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| HIT involves: | IgG antibody to Platelet Factor 4
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| Confirm HIT with: | Serotonin Release Assay
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| Most common drug induced form of thrombocytopenia | HIT
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| HIT type I | Non-immune mediated: 100K platelets, no thrombosis (dc heparin)
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| HIT type II | Immune mediated: 30K platelets, thrombosis risk (require dc heparin, Rx direct thrombin inhibitors & warfarin)
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| HUS tx | D+ HUS: supportive; NO PLTS, NO ABX. Plasmapheresis & FFP
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| IgG antibody to PF4: seen in: | HIT (both art & venous thrombosis)
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| Microangiopathic hemolytic anemia & thrombocytopenia without an identifiable cause = | TTP (also often see neuro sxs)
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| anemia with schistocytes, thrombocytopenia & renal dysfunction in the absence of other causes of coagulopathy = | HUS
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| DIC vs HUS: dx | DIC low fibrinogen; HUS normal fibrinogen
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| Anagrelide may be used for: | P vera; thrombocytosis
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| Bleeding disorder clinical findings: platelet vs coagulopathy | plt: mucosa & skin; petechiae in low plt (NOT plt dysfn); coag: skin & mx; spont hemarthrosis in severe hemophilia
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| most common cause of abnormal bleeding: | thrombocytopenia
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| HUS vs TTP | HUS: no neuro sxs, more renal > TTP
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| Causes of decreased platelet function: membrane defects | Glanzmann Thrombasthenia; Bernard-Soulier
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| Causes of decreased platelet function: Pathway Defects | Arachidonic Pathway Defect
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| Causes of decreased platelet function: medications | Aspirin/Clopidogrel use
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| Causes of decreased platelet function: acquired disease | MDS
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| Diseases causing ITP | SLE, CLL, virus (HIV/Hep C)
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| von W defect is in: | platelet adhesion only
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| HIT occurs when? | 4-10 days post heparin infusion
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| Adult ITP epidemiology | Peak = 20-50 y.o. (2-4 yo in kids). F:M = 2:1
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| ITP etiology | Acquired autoimmune. Isolated thrombocytopenia. Autoantibody binds to platelet membrane antigen -> bound to splenic macrophages & destroyed in spleen -> low platelets 2/2 splenic sequestration & accelerated platelet destruction
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