Intravascular coagulation necrosis of the skin associated with cryofibrinogenemia, diabetes mellitus, and cardiolipin autoantibodies

Zouboulis, Christos C.; Gollnick, Harald; Weber, Sabine; Peter, Hans; Orfanos, Constantin E.

doi:10.1016/0190-9622(91)70275-7

Cited by 18 publications

(4 citation statements)

References 36 publications

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“…Skin biopsy typically shows eosinophilic fibrin thrombi in the superficial and deep dermal blood vessels with minimal inflammation and no vasculitis. 4,5 In a few cases leukocytoclasis, 6 immunoglobulin and complement deposits 7 have also been documented, although these are unusual. Underlying thrombosis is the dominant feature, but immune mechanisms may be contributing since some patients respond to immunosuppression, such as corticosteroids and/or cytotoxic therapy.…”

Section: Discussionmentioning

confidence: 99%

“…Primary cases have been treated with anticoagulation, 6 and corticosteroids and immunosuppressive agents have been used with variable success. 4,5,7 Stanozol (Sanofi Winthrop Pharmaceuticals, New York, NY, USA), an androgen steroid with fibrinolytic activity has also been used successfully in the treatment of this syndrome. 8 There are a few reports of therapeutic plasma exchange in refractory patients.…”

Section: Discussionmentioning

confidence: 99%

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Cryofibrinogenemia and skin necrosis in a patient with diffuse large cell lymphoma after high-dose chemotherapy and autologous stem cell transplantation

Shimoni

Körbling

Champlin

et al. 2000

Bone Marrow Transplant

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Summary:A 34-year-old woman with diffuse mediastinal B cell large cell lymphoma presented 60 days after high-dose chemotherapy and autologous stem cell transplantation, and post-transplant immunotherapy with interleukin-2, with skin necrosis in the ears and extremities. Extensive work-up revealed the presence of cryofibrinogenemia and associated thrombotic vasculopathy. The patient was successfully treated with corticosteroids and therapeutic plasma exchange. However, she had recurrence of large cell lymphoma a few weeks later and died of progressive disease. Cryfibrinogenemia and skin necrosis may have occurred secondary to the imminent relapse, or as a rare complication of high-dose chemotherapy or treatment with interleukin-2. Bone Marrow Transplantation (2000) 26, 1343-1345. Keywords: cryofibrinogenemia; lymphoma; stem cell transplantation; interleukin-2 A 34-year-old woman presented with chest pain and a mediastinal mass. An open biopsy showed diffuse B cell large cell lymphoma. The patient was treated with the CHOP regimen with an initial good response. However shortly after the last cycle, she developed fever, night sweats and recurrent chest pain. She had new axillary adenopathy, as well as re-growth of the mediastinal mass and a left lower lobe lung infiltrate. She received a partial course of mantle radiation therapy with prompt resolution of symptoms. She was then given two courses of ifosfamide and etoposide, and achieved a partial response. Peripheral blood stem cells were collected after the second course. The patient proceeded to high-dose chemotherapy with the BEAM regimen. The stem cells were incubated with interleukin-2 (IL-2) prior to infusion and the patient also day 11 (with sustained ANC Ͼ1 × 10 9 /l for 3 consecutive days) and restaging tests on day 30 showed she was in complete remission. The patient completed the first maintenance course of IL-2 that was given in reduced doses (2 × 10 6 U/m 2 daily by subcutaneous injection, 25% of planned dosing) due to fevers and pancytopenia.She presented 2 weeks later, on day 56 of the transplant, with symptoms of an upper respiratory tract infection and was given oral antibiotics. A few days later she developed fever up to 38.2°C and then rapidly progressive large purpuric/necrotic lesions on her ears, and upper and lower extremities (see Figure 1). The WBC was 3.1 × 10 9 /l, hemoglobin 11.2 g/dl, and platelets 119 × 10 9 /l. A blood smear was normal with no evidence of microangiopathy. Electrolytes, creatinine, and liver function tests were within normal limits. Her LDH was 880 U/l (normal upper limit, 618). There was no laboratory evidence for disseminated intravascular coagulation (DIC). Chest X-ray showed a questionable left lower lobe infiltrate. Blood cultures were negative; serology for acute infection with EBV, CMV, hepatitis viruses and Mycoplasma was negative. PCR for hepatitis C virus RNA was negative. Repeat assays for cryoglobulins were negative. There was a low titer (1:256) of cold agglutinins. Assays of plasma for the presence of cry...

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Cryofibrinogenemia and skin necrosis in a patient with diffuse large cell lymphoma after high-dose chemotherapy and autologous stem cell transplantation

Shimoni

Körbling

Champlin

et al. 2000

Bone Marrow Transplant

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“…3,5,21,22 For example, Blain and associates 5 reported that corticosteroids were ineffective in primary CF (0 out of 3 cases had remission) but were effective in secondary CF (4 out of 5 cases had remission). All the secondary CF effectively treated with corticosteroids in that study were associated with inflammatory or rheumatologic diseases.…”

Section: Discussionmentioning

confidence: 99%

“…3 Treatment for primary CF with reported success includes use of fibrinolytics (eg, streptokinase), 3,[16][17][18][19][20] immunomodulators (eg, glucocorticoids in combination with azathioprine or chlorambucil, and plasmapheresis), 5,[21][22][23][24] and anticoagulants (eg, such as heparin and warfarin). 14,25 Aspirin and colchicine have been shown to be ineffective.…”

Section: Discussionmentioning

confidence: 99%

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Chen

Sreenivasan²,

Shojania³

et al. 2015

Exp Clin Transplant

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Cryofibrinogenemia is a rare disorder in which plasma, not serum, forms a cryoprecipitate. Patients with cryofibrinogenemia may be asymptomatic, or they may have painful ulcers, purpura, livedo reticularis, Raynaud phenomenon, perniosis of the extremities, thrombosis, and arthralgia. Cryofibrinogenemia may be primary or secondary to an underlying disorder such as connective tissue disease, malignancy, infection, drugs, or thromboembolic disease.Here, we present a 41-year-old woman with a pancreatic neuroendocrine tumor who underwent a Whipple procedure in 2003 followed by 2 liver transplants for hepatic metastases. Three years posttransplant, we discovered a biopsy-proven metastatic lesion in her femur. Five years posttransplant, she developed acute, severe pain in both feet, and was found to have cryofibrinogenemia despite immunosuppression posttransplant. Testing for connective tissue diseases and hematologic malignancy were negative. She was treated with high-dose prednisone, which completely resolved her symptoms. We also conducted a review of the literature via a PubMed search to summarize the association of cryofibrinogenemia with malignancy and treating cryofibrinogenemia with corticosteroids.Our study is the first reported case of cryofibrinogenemia that developed secondary to a neuroendocrine tumor posttransplant. Our report suggests that cryofibrinogenemia may occur despite immunosuppression adequate to prevent graft rejection, and that high-dose corticosteroids are an effective treatment for posttransplant cryofibrinogenemia.

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Skin Necrosis

Bonilla-Martinez

Kirsner

2009

Life-Threatening Dermatoses and Emergencies in Dermatology

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Intravascular coagulation necrosis of the skin associated with cryofibrinogenemia, diabetes mellitus, and cardiolipin autoantibodies

Cited by 18 publications

References 36 publications

Cryofibrinogenemia and skin necrosis in a patient with diffuse large cell lymphoma after high-dose chemotherapy and autologous stem cell transplantation

Cryofibrinogenemia and skin necrosis in a patient with diffuse large cell lymphoma after high-dose chemotherapy and autologous stem cell transplantation

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Skin Necrosis

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