Cytophagic Histiocytic Panniculitis Improved by Combined CHOP and Cyclosporin A Treatment.

Abstract

In a 31-year-old Japanese man with cytophagic histiocytic panniculitis (CHP) remission was achieved by a combination of combined chemotherapy CHOPand cyclosporin A treatment. He was admitted to our hospital in January 1994 with recurrent high fever of 40.2°C and tender and violaceous subcutaneous nodules on his trunk, arms and legs. Hedeveloped pancytopenia, hemorrhagic diathesis, liver dysfunction. Histological examination of the biopsied subcutaneous nodule revealed a lobular panniculitis with fat necrosis and a massive infiltration of histiocytes phagocytosing nuclear debris. He was treated initially with 40 mg/day prednisolone. However, following a reduction in prednisolone dosage, his symptoms reappeared. CHOP(cyclophosphamide, doxorubicin, vincristine and prednisolone) therapy was then initiated. Three courses of CHOPtreatment alleviated his symptomsand cyclosporin A was used to maintain his condition for 15 months. His medication was then discontinued and he has been in complete remission for 10 months. Combined treatment of cyclosporin A and CHOPcombined chemotherapy was shown to be effective for this patient with severe CHP. (Internal Medicine 38: 296-301, 1999)

Cytophagic histiocytic panniculitis with haemophagocytosis in a patient with familial multiple lipomatosis and review of the literature

“…Long-term remission on cyclosporin is in contrast with panniculitis in the setting of SPTL, where cyclosporin is ineffective and a short clinical course with early demise is the rule [3,12]. Although initial diagnosis should nowadays be established by appropriate molecular studies to optimise treatment decisions, patients with CHP require close follow-up: cases of initially unapparent lymphoma evolving several years after CHP diagnosis have been reported [3,13].…”

“…Since 1990 the use of combined cytotoxic agents such as cyclophosphamide, doxorubicin, vincristine and prednisolone (CHOP) with or without combination with cyclosporin has seen the mortality rate decrease from 60% to approximately 24% [13], indicating that T-cell specific chemotherapy is optimal treatment for patients with CHP. Complete remission in our case was eventually achieved with the addition of cyclosporin.…”

Cyclosporin in subcutaneous panniculitis-like T-cell lymphoma

“…However, there was no randomized controlled trial due to the rarity of the disease. Subsequent articles also demonstrated successful outcomes using either cyclosporin combined with CHOP or oral cyclosporin alone [10,11]. In one study [11], serum levels of interferon g and soluble interleukin-2 receptor were tremendously elevated during the active disease and normalized after cyclosporin, suggesting that cytokine modulation may be the mechanism of action of this drug.…”

A case of Weber?Christian disease associated with myelodysplastic syndrome

“…Iwasaki et al 16 reported a patient with WCD with increased serum levels of soluble IL-2 receptor, IFN-γ, IL-6, IL-4, and IL-10, who was treated successfully with cyclosporin A. Cytophagic histiocytic panniculitis (CHP) can be a severe variant of Weber-Christian disease characterized by the histopathological appearance of lobular panniculitis infiltrated by T helper cells, and histiocytes containing blood cell fragments, and by a clinical course with marked systemic features, including multiorgan failure, hypertriglyceridemia, and coagulopathy, which may lead to death. [17][18][19] However, cyclosporine A is very effective in CHP. 17,19 From these findings and the case study reported here, it is suggested that T-cell immune responses may be involved in the pathogenesis of WCD.…”

“…[17][18][19] However, cyclosporine A is very effective in CHP. 17,19 From these findings and the case study reported here, it is suggested that T-cell immune responses may be involved in the pathogenesis of WCD. We recommend further study on the pathogenesis of WCD.…”