MULTIPLE MYELOMA COMPLICATED BY FATAL THROMBOTIC THROMBOCYTOPENIC PURPURA (TTP) – A CASE REPORT OF AUTOPSY FINDINGS IN A TERTIARY CARE CENTRE.

Thrombotic thrombocytopenic purpura (TTP) is a syndrome characterized by microangiopathic hemolytic anemia, thrombocytopenia, neurological abnormalities, fever and renal dysfunction. Early clinical suspicion and presumptive diagnosis of TTP helps in timely initiation of treatment modalities speci?c for TTP which may prove to be lifesaving and thus augment in reducing the mortality rate of TTP which is estimated to be 80 – 90 % if left untreated. We report a case of a known case of multiple myeloma who developed TTP which proved fatal despite plasmapheresis. Signi?cant autopsy ?ndings of presence of microthrombi in the microvasculature of multiple organs is also highlighted.

Twenty-one patients with thrombotic thrombocytopenic purpura:a clinical study / 中华急诊医学杂志

Objective To investigate clinical features, outcomes and laboratory findings of thrombotic thrombocytopenic purpura (TTP).Methods Patients with TTP admitted between April 2006 and January 2013 were identified by a retrospective review of records.Totally 21 patients were available,15 females and 6 males,with a median age of 46 years (ranged 18-66).The diagnostic criteria were defined by:(1)thrombocytopenia (<100 ×9 L-1)without other identifiable causes;(2)a negative Coombs'test and hemolytic anemia with schistocytes on the peripheral blood smear;and only those patients meeting the criteria for TTP,both on clinical presentation and their clinical course,were included in this study. Exclusion criteria were:(1)patients discharged or dead within 24 hours after admission;(2)patients treated with plasma exchange therapy in other hospitals;(3)medical data were incomplete;(4)cannot be followed up;and (5 )other causes of thrombotic microangiopathies.General condition of patients,etiology, clinical features,treatment and prognosis were analyzed by using the SPSS 20.0 software.P value of <0.05 was considered as significant.Results Hemolytic anemia and thrombocytopenia appeared in all of the patients. Twelve patients (57.2%) had the classical pentad manifestations of TTP (fever, thrombocytopenia,microangiopathic hemolytic anemia,symptoms of nervous system,renal injury),seven patients (33.3%)had tetrad of TTP clinical manifestations (thrombocytopenia,microangiopathic hemolytic anemia,symptoms of nervous system,fever or renal injury),and only two patients showed the triad manifestations of TTP (thrombocytopenia, microangiopathic hemolytic anemia, symptoms of nervous system).In our studies,seven patients accepted plasmapheresis,and five of them (71.4%)achieved remission.Conclusions TTP progresses quickly.Plasmapheresis is still the treatment of choice for TTP patients.Etiological treatment can help to control the conditions of patients with TTP.

Comparison of Coagulation Factor Activities of Cryoprecipitate-depleted plasma(CDP) Prepared by Two Different Methods / 대한수혈학회지

BACKGROUND: Cryoprecipitate depleted plasma(CDP) that is selectively used for therapeutic plasma exchange for the thrombotic thrombocytopenic purpura(TTP). We evaluated coagulation factor activities of CDP prepared by two different methods to use CDP as a blood component and induce a new guideline. METHODS: We studied 32 units of CDP were made from FFP collected from 16 donors. To prepare CDP, units of FFP were thawed in a 4degrees C water bath for 1 to 2 hours(rapid thawing) or thawed in a 1 to 6degrees C refrigerator for 14 to 16 hours(overnight thawing). Then CDP were refrozen within 1 hours, stored at -45degrees C and re-thawed in three weeks later. We measured prothrombin time(PT), activated partial prothrombin time(aPTT), fibrinogen, coagulation factor V, VIII, von willebrend factor(vWF) and vWF multimer of CDP at the time of preparation, after re-freezing and re-thawing. And we compared them with the results of platelet poor plasma(PPP). RESULTS: The mean volume of CDP was 129+/-12mL. We found all measured factors of CDP except factor V were significantly lower or longer than those of PPP. In the comparision of thawing method, rapid thawing CDP showed longer PT, higher activities of fibrinogen and factor VIII, lower activities of vWF than those of overnight thawing with no significant change of factor V. No significant changes were notified in all factors between the results of CDP within 1 hour of preparation and those of after re-freezing and re-thawing. CONCLUSIONS: CDP prepared by rapid thawing is more recommendable for therapeutic plasma exchange for TTP. CDP prepared by re-freezing and re-thawing can be used as a CDP just prepared from FFP.

A Case of Systemic Lupus Erythematosus Associated with Thrombotic Thrombocytopenic Purpura / 대한신장학회잡지

Although thrombotic thrombocytopenic purpura (TTP) has been described in patients with systemic lupus erythematosus (SLE), the relationship between these two diseases is controversial. It is reported that survival in SLE associated with TTP correlated with the use of plasma exchange rather than with the activity of the underlying autoimmune disease. We experienced a patient with SLE who developed TTP. The patient temporarily responded to therapy including aggressive plasmapheresis with infusion of fresh frozen plasma. Plasmapheresis could not be continued because of deterioration of renal function and pulmonary edema. TTP recurred and the patient died.