Successful treatment of thrombotic thrombocytopenic purpura with repeated plasma exchange in a patient with microscopic polyangitis

Abstract

Thrombotic thrombocytopenic purpura (TTP) is in rare cases associated with antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis, and often has a fatal outcome. We report the case of a 77-year-old woman with microscopic polyangitis (MPA) presenting with TTP. Rapidly progressive renal dysfunction and paralysis and sensory disturbance of the left lower limb were noted. Serum creatinine was 3.95 mg/dl, and the titer of myeloperoxidase-ANCA was 238 EU. She was diagnosed with MPA, and high-dose methylprednisolone was initiated, followed by 60 mg/day of prednisolone. Hemolytic anemia with red blood cell fragmentation, purpura, and thrombocytopenia developed during the course of active MPA. The activity of disintegrin and metalloproteinase with thrombospondin type 1 motifs 13 (ADAMTS13) was moderately decreased (27%). She was diagnosed with TTP, and plasma infusion was initiated, followed by plasma exchange (PE) with 40 units of fresh frozen plasma. Thrombocytopenia continued for more than a month (5-10x10(4)/microl). PE was repeatedly performed two or three times a week during the first 8 weeks from the beginning of PE in addition to prednisolone. Her clinical and laboratory findings gradually improved, and ADAMTS13 activity increased to 68%. The findings in this case suggested that ANCA-associated vasculitis may be involved in the development and the pathogenesis of TTP, and that repeated PE may need to be performed in addition to immunosuppressive therapy.

Clinicopathologic Characteristics and Outcomes of Renal Thrombotic Microangiopathy in Anti-Neutrophil Cytoplasmic Autoantibody-Associated Glomerulonephritis

“…However, the prevalence in other countries is unclear, especially for renal TMA in ANCA-associated GN. As reported in previous case reports, patients with concomitant renal TMA in ANCA-associated GN presented with more severe renal injury (3)(4)(5)(6)(7)(8)(9). Patients with renal TMA in our cohort also had a higher level of initial serum creatinine, higher percentage of cellular crescents and fibrinoid necrosis, and more severe interstitial infiltration compared with patients without TMA, which, by a large cohort study, further extends previous findings.…”

“…intensive immunosuppressive therapy, such as plasma exchange, as described in previous case reports (3)(4)(5)(6)(7)(8).…”

“…To our knowledge, TMA in ANCA-associated GN has only been reported in isolated case reports (3)(4)(5)(6)(7)(8)(9), with two patients showing pathologic features of renal TMA. The clinicopathologic characteristics, especially the prognostic value of pathologic findings of renal TMA, in ANCA-associated GN are far from clear.…”

Systemic Lupus Erythematosus (SLE) with Acute Nephritis, Antineutrophil Cytoplasmic Antibody- (ANCA-) Associated Vasculitis, and Thrombotic Thrombocytopenic Purpura (TTP): A Rare Case Report with Literature Review

“…Mortality was less with PEX therapy alone and least with PEX and immunosuppressants [9]. In cases of TTP with vasculitis, all cases were treated with at least PEX and glucocorticoids, with all cases achieving clinical remission [6,[12][13][14]. Clinical judgement and supporting lab results must be used to rule out an infectious process prior to initiating immunosuppressive therapy.…”

“…A literature search using the databases Pubmed, Web of Science, Cochrane, and Google Scholar identified 4 case reports [6,[12][13][14] on patients presenting with TTP and vasculitis, an uncommon association.…”