primary myelofibrosis/edema

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Castleman-Kojima disease (TAFRO syndrome) : a novel systemic inflammatory disease characterized by a constellation of symptoms, namely, thrombocytopenia, ascites (anasarca), microcytic anemia, myelofibrosis, renal dysfunction, and organomegaly : a status report and summary of Fukushima (6 June, 2012) and Nagoya meetings (22 September, 2012).

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Recently, a unique clinicopathologic variant of multicentric Castleman's disease (MCD) has been identified in Japan. This disease is characterized by a constellation of symptoms, as listed in the title, and multiple lymphadenopathy of mild degree with a pathologic diagnosis of atypical CD, often

A case of multicentric castleman's disease of mixed-type, which showed constellation of symptoms, i.e., thrombocytopenia, anasarca, anemia, fever, myelofibrosis, and lymphadenopathy.

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[Pseudo-adult Still's disease, anasarca, thrombotic thrombocytopenic purpura and dysautonomia: An atypical presentation of multicentric Castleman's disease. Discussion of TAFRO syndrome].

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BACKGROUND Multicentric Castleman's disease can mimic adult-onset Still disease. It is exceptionally associated with anasarca, thrombotic microangiopathy and dysautonomia. METHODS We report a 32-year-old woman with an association of oligoanuria, anasarca, thrombotic microangiopathy with features

Focal segmental glomerulosclerosis associated with idiopathic myelofibrosis.

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A patient with idiopathic myelofibrosis with nephrotic syndrome is reported. Seven months after the initial diagnosis of myelofibrosis, the patient was presented with dyspnea, generalized edema, heavy proteinuria, massive pleural effusion, and ascites. Renal biopsy showed focal segmental

[Ruxolitinib combined with prednisone, thalidomide and danazol for treatment of myelofibrosis: a pilot study].

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Objective: To evaluate the efficacy and tolerability of ruxolitinib combined with prednisone, thalidomide and danazol for treatment of in myelofibrosis (MF). Methods: Patients of MF according to the WHO 2016 criteria, received ruxolitinib (RUX) combined with prednisone, thalidomide and

Phase 2 trial of imatinib mesylate in myelofibrosis with myeloid metaplasia.

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In a phase 2 study, 23 patients with myelofibrosis with myeloid metaplasia were treated with imatinib mesylate at a constant dose of 400 mg/d. Treatment was held in 16 patients (70%), after 1 to 12 weeks, because of side effects (neutropenia, 6 patients; musculoskeletal pain, 5 patients;

Endolymphatic hydrops as a cause of audio-vestibular manifestations in relapsing polychondritis.

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Relapsing polychondritis (RP) is characterized by inflammation and subsequent degeneration of cartilage. We report a 61-year-old woman who had RP with audio-vestibular manifestations. She was also diagnosed as having a myelofibrosis with myeloid metaplasia (MMM). Bilateral endolymphatic hydrops (EH)

Autoimmune Myelofibrosis in Systemic Lupus Erythematosus Report of Two Cases and Review of the Literature.

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Autoimmune myelofibrosis (AIMF) is a rare entity of steroid-responsive bone marrow fibrosis that accompanies a variety of autoimmune diseases, particularly systemic lupus erythematosus (SLE). Rarely it may occur in patients with autoimmune markers but no definable autoimmune disease (Primary-AIMF).

A pilot study of recombinant human interleukin-4 therapy of myelofibrosis.

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Twelve patients with myelofibrosis were treated with recombinant human interleukin-4 (IL-4) administered subcutaneously thrice weekly. Dosage ranged from 1 microg/kg to 4 microg/kg. Median patient age was 65 years (range 36-74). Five patients had transient minor responses, and 5 patients had

Successful treatment of a patient with multicentric Castleman's disease who presented with thrombocytopenia, ascites, renal failure and myelofibrosis using tocilizumab, an anti-interleukin-6 receptor antibody.

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We herein describe an unusual case of multicentric Castleman's disease accompanied by thrombocytopenia, ascites, renal failure and myelofibrosis in a Japanese woman. The patient was initially diagnosed as having myelodysplastic syndrome with myelofibrosis. The general condition of the patient

Thalidomide therapy for myelofibrosis with myeloid metaplasia.

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BACKGROUND Thalidomide is a putative antiangiogenesis agent with activity in several hematologic malignancies. METHODS Forty-four patients who had myelofibrosis with myeloid metaplasia received treatment with thalidomide in a Phase II clinical trial at a dose of 200 mg daily with escalation by 200

Acute duodenal ischemia and periampullary intramural hematoma after an uneventful endoscopic retrograde cholangiopancreatography in a patient with primary myelofibrosis.

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Acute duodenal ischemia and periampullary intramural hematoma are rare complications after endoscopic retrograde cholangiopancreatography (ERCP). A 77-year-old man with splenomegaly complained of abdominal pain caused by common bile duct (CBD) stone. After successful removal of the CBD stone without

Therapy-related changes of the bone marrow in chronic idiopathic myelofibrosis.

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In chronic myeloproliferative disorders (CMPDs) a conflict of opinion exists regarding therapy-induced bone marrow (BM) changes and the evolution of myelofibrosis during the lengthy course of the disease. For a more elaborate study of these features chronic idiopathic myelofibrosis (IMF) seems to be

Amyloidosis complicating idiopathic myelofibrosis.

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An 84-year-old man presented with ankle edema, significant proteinuria, and mild impairment of renal function soon after treatment was started for idiopathic myelofibrosis. Renal amyloidosis was found on biopsy. The amyloid deposit was resistant to potassium permanganate treatment and showed no

Unexplained cause of thrombocytopenia, fever, anasarca and hypothyroidism: TAFRO syndrome with thrombotic microangiopathy renal histology

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TAFRO (thrombocytopenia, anasarca, fever, reticulin myelofibrosis or renal dysfunction and organomegaly) syndrome is a systemic inflammatory disease characterised by thrombocytopenia, anasarca, fever or inflammatory syndrome, reticulin myelofibrosis or renal dysfunction and organomegaly. It was

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