Immune thrombotic thrombocytopenic purpura in older patients: Results from the Spanish TTP Registry (REPTT).

Immune thrombotic thrombocytopenic purpura (iTTP) is an ultra-rare disease that seldom occurs in the elderly. Few reports have studied the clinical course of iTTP in older patients. In this study, we have analysed the clinical characteristics at presentation and response to therapy in a series of 44 patients with iTTP ≥60 years at diagnosis from the Spanish TTP Registry and compared them with 209 patients with <60 years at diagnosis from the same Registry. Similar symptoms and laboratory results were described in both groups, except for a higher incidence of renal dysfunction among older patients (23% vs. 43.1%; p = 0.008). Front-line treatment in patients ≥60 years was like that administered in younger patients. Also, no evidence of a difference in clinical response and overall survival was seen in both groups. Of note, 14 and 25 patients ≥60 years received treatment with caplacizumab and rituximab, respectively, showing a favourable safety and efficacy profile, like that observed in patients <60 years.

ADAMTS-13 activity and von Willebrand factor levels in methylene-blue photo-inactivated plasma processed by either the Springe method or an 'in house' system.

BACKGROUND AND OBJECTIVES: Methylene blue photo-inactivated plasma (MBPIP) has been reported to be less effective than fresh-frozen plasma (FFP) in the treatment of thrombotic thrombocytopenic purpura, which suggests a reduced content of the von Willebrand factor metalloprotease ADAMTS-13 in MBPIP. MATERIALS AND METHODS: ADAMTS-13 activity and von Willebrand factor antigen (vWF:Ag) levels were measured in plasma before and after photo-oxidation by either the Springe method or a commercial 'in house' system as well as in cryoprecipitate-poor plasma (CPP) and FFP (20 units each). RESULTS: Levels of ADAMTS-13 activity in MBPIP processed by the Springe method or the commercial 'in house' system were comparable to one another and did not significantly differ from levels found in FFP [median (range): 114% (57-139%), 99% (74-123%), and 106% (70-130%), respectively]. ADAMTS-13 activity was significantly reduced in CPP [median (range): 87% (70-107%) as compared with FFP (P < 0.05). Levels of vWF:Ag decreased after photo-oxidation by both methods. CONCLUSION: In vitro ADAMTS-13 activity was conserved in MBPIP processed by the two photo-oxidation methods analysed and did not significantly differ from levels found in FFP.

[Asymmetric polyarthritis in Sézary's syndrome]. / Poliartritis asimétrica en el síndrome de Sézary.

We present the case of a patient with negative serology asymmetric polyarthritis and erythematous-squamous cutaneous lesions of two years evolution, initially attributed to psoriasis, which histopathology (hyperkeratosis, epidermic infiltration due to atypical CD4+ lymphocytes with formation of Pautrier's microabscesses) together with hematological findings (persistent lymphocytosis with cells with cerebriform nucleus, 97% CD4+) allowed the diagnosis of Sézary's syndrome. To our knowledge, this is the eight documented case of arthritis in Sézary's syndrome and the first one with asymmetric chronic polyarthritis.