Pearls from Dr. Andrew Eisenberger
Typical, sporadic form of TTP: auto-antibodies against ADAMTS-13 neutralize this vWF multimer protease --> multimer accumulation --> abnormal platelet adhesion and activation --> systemic microvascular aggregation of platelets --> CNS and other organ ischemia
- Drug related (endothelial damage NOT related to ADAMTS-13): cyclosporine, gemcitabine
- Stroke in TTP = usually microangiopathic but can be medium- and large-vessel related
Posted 05/29/16 12:24:46 PM by Matthew Cummings
Microangiopathic hemolytic anemia (schistocytes + ↑LDH/indirect bili) AND thrombocytopenia without an alternative apparent cause? Strongly consider TTP
- Quick way to look for hemolysis is UA – check for presence of hemoglobin without RBCs
- Differential: Sepsis, Malignancy, Severe HTN, HELLP/pre-eclampsia, APLS, Scleroderma crisis
Posted 05/29/16 12:25:44 PM by Matthew Cummings
Low threshold for plasma exchange if unexplained MAHA + thrombocytopenia (even if diagnosis not definitive), continue until platelets >150,000
- Placing a central line for PLEX in patients with TTP and marked thrombocytopenia: do not give platelets unless actively bleeding, give FFP, and ensure the most experienced clinicians are doing the procedure (note: can give platelets in ITP)
- If CNS symptoms or decompensation before initiation of PLEX --> FFP (replace ADAMTS13)
- Must send assay for ADAMTS-13 and inhibitor titer before giving FFP (usually used for prognosis rather than to guide therapy given prolonged turnaround time and need for immediate treatment)
Posted 05/29/16 12:26:53 PM by Matthew Cummings
Severe/refractory TTP? Consider adjunctive therapies: glucocorticoids, rituximab
Posted 05/29/16 12:27:45 PM by Matthew Cummings
For further reading...
Posted 05/29/16 12:30:29 PM by Matthew Cummings
Prednisone for ITP with decrease production of anti-platelet antibodies. However, half life of IgG is 3 weeks, so immediate response to Prednisone is not this mechanism. Prednisone also decreases expression of FcGamma receptor, which leads to fast response seen with steroids. IVIG saturates the FcGamma receptor, which prevents opsonization of platelets coated with antibodies.
Posted 05/24/17 09:43:43 AM by Emily Miller
Hematologic Complications of Bariatric Surgery: B12 deficiency, Iron deficiency, Copper deficiency - causes dorsal column neuropathy, anemia and neutropenia.
Posted 05/24/17 09:45:40 AM by Emily Miller
A shortened PTT can be a risk factor for thrombosis. Can see with conditions that cause increased fibrinogen, factor VIII levels or von willebrand factor.
Posted 05/24/17 09:47:16 AM by Emily Miller
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It is not meant to replace the advice of a health professional.