A case of mixed connective tissue disease successfully treated for hemophagocytic syndrome with intermittent intravenous injection of cyclophosphamide

Masashi Kato, Shinji Sato, Misako Suzuki, Hiroko Oka, Yuko Kaneko, Hidekata Yasuoka, Takaki Nojima, Akira Suwa, Michito Hirakata, Yasuo Ikeda

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5 Citations (Scopus)

Abstract

A 52 year-old woman noticed general fatigue, polyarthralgia, and muscle weakness of lower extremities in October 2001. In December, she felt difficulty in walking due to muscle weakness. In January 2002, she admitted another hospital because of dyspnea on exertion and edema of lower extremities. Laboratory test revealed leukocytopenia, the elevation of creatine kinase and positive anti-U1-RNP antibodies. Her chest computed tomography (CT) showed severe interstitial pneumonia. Cardiac echogram revealed that she had pericardial effusion and pulmonary hypertension. Then she was transferred to Keio University Hospital and she was diagnosed as having mixed connective tissue disease (MCTD) manifestating myositis, interstitial pneumonia, pulmonary hypertension and pericarditis. Prednisolone (PSL) 60mg daily following to methylprednisolone (mPSL) pulse therapy was begun and her symptoms were gradually improved. In middle of February, she complained of high fever over 39.0 degrees C. Bacterial culture tests were negative and laboratory data indicated pancytopenia and a high level of serum ferritin. Bone marrow aspiration revealed hemophagocytosis in bone marrow specimens and she was diagnosed as having hemophagocytic syndrome associated with MCTD. mPSL pulse therapy was not effective and intermittent cyclophosphamide pulse therapy (IV-CY) was performed resulting in improvement of the symptoms. This case suggested the effectiveness of IV-CY therapy in patients with corticosteroid-resistant HPS associated with connective tissue diseases.

Original languageEnglish
Pages (from-to)345-349
Number of pages5
JournalNihon Rinshō Men'eki Gakkai kaishi = Japanese journal of clinical immunology
Volume27
Issue number5
Publication statusPublished - 2004

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Mixed Connective Tissue Disease
Hemophagocytic Lymphohistiocytosis
Intravenous Injections
Cyclophosphamide
Interstitial Lung Diseases
Muscle Weakness
Methylprednisolone
Pulmonary Hypertension
Lower Extremity
Bone Marrow
Mobility Limitation
Pancytopenia
Myositis
Connective Tissue Diseases
Pericarditis
Pericardial Effusion
Leukopenia
Arthralgia
Therapeutics
Ferritins

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A case of mixed connective tissue disease successfully treated for hemophagocytic syndrome with intermittent intravenous injection of cyclophosphamide. / Kato, Masashi; Sato, Shinji; Suzuki, Misako; Oka, Hiroko; Kaneko, Yuko; Yasuoka, Hidekata; Nojima, Takaki; Suwa, Akira; Hirakata, Michito; Ikeda, Yasuo.

In: Nihon Rinshō Men'eki Gakkai kaishi = Japanese journal of clinical immunology, Vol. 27, No. 5, 2004, p. 345-349.

Research output: Contribution to journalArticle

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AU - Suzuki, Misako

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AU - Kaneko, Yuko

AU - Yasuoka, Hidekata

AU - Nojima, Takaki

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AU - Hirakata, Michito

AU - Ikeda, Yasuo

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AB - A 52 year-old woman noticed general fatigue, polyarthralgia, and muscle weakness of lower extremities in October 2001. In December, she felt difficulty in walking due to muscle weakness. In January 2002, she admitted another hospital because of dyspnea on exertion and edema of lower extremities. Laboratory test revealed leukocytopenia, the elevation of creatine kinase and positive anti-U1-RNP antibodies. Her chest computed tomography (CT) showed severe interstitial pneumonia. Cardiac echogram revealed that she had pericardial effusion and pulmonary hypertension. Then she was transferred to Keio University Hospital and she was diagnosed as having mixed connective tissue disease (MCTD) manifestating myositis, interstitial pneumonia, pulmonary hypertension and pericarditis. Prednisolone (PSL) 60mg daily following to methylprednisolone (mPSL) pulse therapy was begun and her symptoms were gradually improved. In middle of February, she complained of high fever over 39.0 degrees C. Bacterial culture tests were negative and laboratory data indicated pancytopenia and a high level of serum ferritin. Bone marrow aspiration revealed hemophagocytosis in bone marrow specimens and she was diagnosed as having hemophagocytic syndrome associated with MCTD. mPSL pulse therapy was not effective and intermittent cyclophosphamide pulse therapy (IV-CY) was performed resulting in improvement of the symptoms. This case suggested the effectiveness of IV-CY therapy in patients with corticosteroid-resistant HPS associated with connective tissue diseases.

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