Scleroderma associated with thrombocytopenia and Coombs-positive hemolytic anemia

Ivey, Kevin J.; Hwang, Yun-Fei; Sheets, Raymond F.

doi:10.1016/0002-9343(71)90310-x

Cited by 23 publications

(13 citation statements)

References 4 publications

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“…In 2 of our patients, no cause of the thrombocytopenia was found, and whether these resemble the recently described cases of scleroderma and autoimmune thrombo cytopenia [9] remains speculative. Likewise, thrombocytosis indicates a serious concur rent disease such as arteritis or tumor.…”

Section: Discussionmentioning

confidence: 48%

Hematological Abnormalities in Scleroderma

Frayha¹,

Shulman²,

Stevens³

1980

Acta Haematol

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Hematological abnormalities in scleroderma indicate a specific complication of the disease itself, or an associated illness. Among 180 patients with scleroderma, anemia was detected in 25% and was attributed to chronic inflammatory disease (usually an overlap syndrome), bleeding mucosal telangiectases as part of the CREST syndrome, intestinal malabsorption, and microangiopathic hemolysis. Leukocytosis, present in 14%, was correlated with active myopathy and/or advanced visceral involvement while leukopenia was suggestive of a crossover with systemic lupus erythematosus. Thrombocytopenia was often a manifestation of microangiopathy, and thrombocytosis was associated with an arteritis or a tumor syndrome.

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Section: Discussionmentioning

confidence: 48%

Hematological Abnormalities in Scleroderma

Frayha¹,

Shulman²,

Stevens³

1980

Acta Haematol

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“…There have been a few reports [3, 4, 5, 6, 7, 8, 9]of scleroderma with bone marrow hypofunction, one of which was due to the side effects of D- penicillamine [9]. In addition, there have been a few reports of scleroderma with autoimmune hemolytic anemia [10, 11, 12, 13, 14], autoimmune thrombocytopenia [13, 15]and/or autoimmune leukopenia [16]. …”

Section: Discussionmentioning

confidence: 99%

Scleroderma Associated with Anemia and Thrombocytopenia that Responded Well to Cyclosporin

Kamada

Kobayashi²,

Katada³

et al. 2000

Acta Haematol

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A 55-year-old woman came to our hospital because of cutaneous sclerosis of the limbs in September 1996, and was diagnosed with scleroderma based on a skin biopsy. In August 1997, the cutaneous sclerosis became progressive (hemoglobin level, 4.3 g/dl; platelet count, 7 × 10⁹/l). The laboratory results were positive for the direct Coombs test, bone marrow aspiration showed a dry tap, and the bone marrow biopsy showed marked fatty marrow. Indium-111 bone marrow scintigraphy showed a markedly decreasing uptake. These findings indicated bone marrow hypoplasia associated with hemolytic anemia. After prednisolone therapy (60 mg) was initiated, the direct Coombs test became negative but the blood cell count did not increase. Then, 300 mg of cyclosporin was initiated and anemia and thrombocytopenia improved. The cyclosporin dosage was gradually decreased and the patient’s hematological condition was good, although the cutaneous sclerosis changed only a little. This is a rare and interesting case of a patient with scleroderma associated with bone marrow insufficiency and hemolysis who responded well to cyclosporin.

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“…Thrombocytopenia is a rare but recognized compli cation of progressive systemic sclerosis (PSS) (1)(2)(3)(4). Although immune mechanisms have been suggested to be partly responsible for thrombocytopenia in PSS, platelet-associated IgG (PAIgG) has not been assayed previously (1)(2)(3)(4)).…”

Section: Introductionmentioning

confidence: 99%

“…Although immune mechanisms have been suggested to be partly responsible for thrombocytopenia in PSS, platelet-associated IgG (PAIgG) has not been assayed previously (1)(2)(3)(4)). This appears to be the first case report of D-penicillamine-induced immune thrombocytopenia developing in a patient with PSS.…”

Section: Introductionmentioning

confidence: 99%

“…The results of the test are summarized in Table 2. Discussion Thrombocytopenia is a rare complication of PSS (1)(2)(3)(4). Three etiological elements have been suggested: 1) complication of Coombs positive hemolytic anemia and/or SLE (1, 2), 2) microangiopathic hemolytic anemia secondary to scleroderma kidney (2), 3) inci dental combination of idiopathic thrombocytopenic purpura (1 -4).…”

Section: Introductionmentioning

confidence: 99%

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Progressive Systemic Sclerosis Complicated with Immune Thrombocytopenia during D-penicillamine Therapy.

et al. 1992

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Immune thrombocytopenia is a rare complication of progressive systemic sclerosis (PSS). A 47-year-old female with PSS treated with D-penicillamine developed immune thrombocytopenia, which promptly responded to prednisolone and withdrawal of D-penicillamine. Platelet-associated IgG was elevated and the bone marrow megakaryocyte count was normal. There was an inverse relationship between the level of platelet-associated IgG and the platelet count. A lymphocyte stimulation test sensitized by D-penicillamine was positive. The present case suggests that immune thrombocytopenia may be regarded as one of the D-penicillaminerelated immune abnormalities. To our knowledge, its association with PSS has never been reported. (Internal Medicine 31: 244-245, 1992)

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Scleroderma associated with thrombocytopenia and Coombs-positive hemolytic anemia

Cited by 23 publications

References 4 publications

Hematological Abnormalities in Scleroderma

Hematological Abnormalities in Scleroderma

Scleroderma Associated with Anemia and Thrombocytopenia that Responded Well to Cyclosporin

Progressive Systemic Sclerosis Complicated with Immune Thrombocytopenia during D-penicillamine Therapy.

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