Background and Purpose
Thrombotic thrombocytopenic purpura (TTP) is a heterogeneous disease primarily characterized by thrombocytopenia and microangiopathic hemolytic anemia. Therapeutic plasma exchange has dramatically improved mortality, allowing for emergence of refractory, relapsing, and atypical presentations.
in this case series we aim to present our institutional data for Apheresis in Sheikh khalifa medical City in AbuDhabi.
We will also present patient demographic and clinical presentation and treatment protocol we use
Methodology
-Case series with Retrospective review.
-Routine laboratory tests such as peripheral blood cell counts, reticulocyte count, coagulation profile, serum lactate dehydrogenase (LDH), bilirubin, serum creatinine, cardiac enzymes, and urinalysis, were performed.
-ADAMTS13 levels and inhibitor titer were determined for all patient in outside lab
-Baseline demographic characteristics were calculated in frequencies and percentages. (include age ,Gender , clinical manifestations and treatment strategy)
Results and Discussions
thrombotic thrombocytopenic purpura (TTP) pentad consisting of fever, thrombocytopenia, microangiopathic hemolytic anemia (MAHA), neurological abnormalities, and renal failure. less than 5 % of patient reported in literature have all associated clinical features.
-Total of 10 patients M:F 4:2 , Median Age 44yr 50% presented with Neurological manifestations and renal disease , 30% presented with Fever only 20% had cardiac manifestation on admission . None of the patient presented with all 5 pentad.
-All patients received TPE , steroid .
-90 % of the patients received Rituximab except for 1 because of Allergy.
-All patients has low ADAMTS 13 , except one has normal ADAMTS13 but came with relapse and on first admission had low ADAMTS13
-All patient presented with MAHA and TCP except 2 patient whom had normal Hb but significant schistocytes on peripheral blood with TCP both patient where relapsed cases.
-3 patient were relapsed 7 de novo , the 3 relapsed cases all did not receive Rituximab in first remission . One of them relapsed twice but did not received Rituximab due to allergy
-Although some publication include large number of TTP patients, but only few case reports have evaluated the clinical feature, laboratory parameters and therapeutic outcome of TTP.
Without treatment, TTP is almost uniformly fatal with a mortality rate approaching 90%. With the timely institution of therapeutic plasma exchange (TPE) mortality decreases to about 10%-20%.
A disintegrin and metalloprotease with thrombospondin Type 1 motif, Member 13 (ADAMTS13) levels less than 5% are a hallmark of TTP.
We do ADAMTS 13 Activity and inhibitor titre levels in outside facility TAWAM hospital with turn-around time of 7 days which is helpful in planning Rituximab treatment.
with availability of Rituximab our relapse rates are low but not zero
Conclusions
-Thrombotic thrombocytopenic purpura (TTP) pentad consisting of fever, thrombocytopenia, microangiopathic hemolytic anemia (MAHA), neurological abnormalities, and renal failure.
-5 % of patient reported in literature have all associated clinical features.
-We found that majority of patient presented with evidence of thrombocytopenia and MAHA only.
-Without treatment, TTP is almost uniformly fatal with a mortality rate approaching 90%. With the timely institution of therapeutic plasma exchange (TPE) mortality decreases to about 10%-20%.
-TPE ,steroid and rituximab was very effective in achieving sustain remission in 100% of ours patients with median follow up 8 month
-More awareness is needed for early diagnosis and early referral to centers with appropriate tertiary care facilities.
Figure
Disclosures
No relevant conflicts of interest to declare.
Microangiopathic Hemolytic Anemia and Fulminant Renal Failure: A Rare Manifestation of Pheochromocytoma
