Hemophagocytic syndrome in a patient with rheumatoid arthritis

Abstract

A 76-year-old man with rheumatoid arthritis, who had been treated with oral prednisolone and methotrexate, presented with high fever and generalized fatigability. Laboratory data demonstrated marked pancytopenia, which we first regarded as a side effect of methotrexate, and leucovorin was administered with granulocyte-colony stimulating factor and transfusions. Because no recovery was recognized, however, bone marrow aspiration was performed, by which hemophagocytic syndrome was diagnosed. After corticosteroid pulse therapy was initiated, the patient’s symptoms were rapidly attenuated and laboratory data rapidly normalized.     

More than skin deep

We present the case of a woman in her 50s with past medical history significant for psoriasis treated with methotrexate on a stable dose for the past 20?years, diabetes mellitus and chronic kidney disease. In the setting of a long flight, dehydration and non steroidal anti-inflammatory drug consumption, the patient presented to the emergency department with oral mucositis and cutaneous erosions and ulcers of the psoriasis plaques. MTX levels were normal corroborated by three different measurements in 24?h. Initially the complete blood count tests were significant for macrocytic, thrombocytopenia (82.000 10³/L) and impaired kidney function. The patient was diagnosed of acute methotrexate toxicity and started on intravenous folinic acid. In 24?h the patient developed severe pancytopenia. She required treatment with colony-stimulating factors, platelet and blood transfusions. After 10?days, the CBC improved to normal levels and the cutaneous lesions resolved.     

Molecular Diagnosis of Shwachman-Diamond Syndrome Presenting with Pancytopenia at an Early Age: The First Report from Turkey

A three-month-old boy presented with growth failure, skeletal abnormalities, otitis media and pancytopenia. Exocrine pancreatic insufficiency was confirmed by low levels of fecal elastase. He was diagnosed as Shwachman-Diamond syndrome by clinical and laboratory findings. The diagnosis was confirmed by sequence analysis for SBDS gene on chromosome seven revealing compound heterozygous mutation, which are c.258+2T-C and c.183-184TA-CT. Matched unrelated donor screening for hematopoietic stem cell transplantation was initiated. Unfortunately, he died of respiratory difficulty at 5 months of age. Our case is the youngest patient whose presumptive Shwachman-Diamond syndrome diagnosis was confirmed by molecular analysis.     

Aplastic anemia complicating systemic lupus erythematosus: successful management with cyclosporine

Anemia is common with connective tissue disorders, but pancytopenia is rare. We report a 22-year-old female who presented with menorrhagia, seizures, anemia, leukocytosis, thrombocytopenia, pericardial effusion, positive ANA, and evidence of vasculitis on CT head scan and was diagnosed with systemic lupus erythematosus (SLE). After 7 months of remission, she was readmitted with menorrhagia and pancytopenia. Investigations revealed aplastic anemia. She survived on transfusion support for 6 weeks, during which period she received methylprednisolone and cyclophosphamide pulses, and phenytoin was omitted but to no avail. Cyclosporine (300 mg/day) was started and the aplastic anemia responded. After 4 months of therapy, the cyclosporine was gradually tapered over the next 2 months. The patient has been on 10 mg/day of prednisolone for the last 6 months. Aplastic anemia is rare in SLE and the response to immunosuppressants is variable, but here is a success story.     

Polyarteritis nodosa presenting as pancytopenia: case report and review of the literature

Polyarteritis nodosa (PAN) is not commonly associated with hematologic abnormalities. We report the first case of pancytopenia as the presenting symptom of PAN. There was no associated malignancy or reactive hemophagocytic syndrome (RHS) to explain the findings. The patient, after successful treatment of PAN, subsequently developed RHS a few years later. He is the fourth reported case of RHS in a patient with PAN. In addition to describing the patient's presentation and clinical course, a systematic review of the literature was performed examining the association between hematologic conditions, including RHS, and PAN.     

Visceral Leishmaniasis mimicking a flare of systemic lupus erythematosus

Summary Fever in systemic lupus erythematosus (SLE) may be caused by exacerbation of the disease itself or by infection. We report on a patient with a long standing history of SLE that was complicated by fever and pancytopenia with no splenomegaly. SLE disease activity was suspected because of an elevated DNA-antibody titer. The early positive response to corticoid therapy may have masked the underlying infection. Visceral leishmaniasis was diagnosed by a repeated bone marrow biopsy and serological testing.     

骨髓增生活跃的特殊类型全血细胞减少患者应用糖皮质激素疗效分析

目的 观察对骨髓增生活跃、网织红细胞计数＞1%、排除白血病等可引起全血细胞减少的疾病,及无足够证据诊断免疫相关性全血细胞减少和溶血性贫血的临床难于诊治的全血细胞减少患者给予应用糖皮质激素的早期疗效.方法 回顾分析12例符合上述条件患者应用糖皮质激素治疗的效果.结果 患者外周血白细胞、中性粒细胞绝对值、血红蛋白浓度有明显...     

Visceral leishmaniasis in hematopoietic cell transplantation: Case report and review of the literature

Visceral leishmaniasis has been recognized as an opportunistic infection affecting people with cellular-immunity impairment, including hematopoietic cell transplantation (HCT) recipients. We describe the case of a young Italian man with Hodgkin lymphoma, who developed visceral leishmaniasis after multiple lines of chemotherapy and allogenic HCT. Literature review of visceral leishmaniasis in HCT recipients was also performed. Eleven patients (median age 50 years, 9 male) developed visceral leishmaniasis after allogenic (n = 9) and autologous (n = 2) HCT. Most of them presented with fever and pancytopenia. Bone marrow examination was the main diagnostic technique; liposomal amphotericin B was the treatment of choice. Four out of eight patients (for whom data are available) experienced visceral leishmaniasis relapse. Visceral leishmaniasis in HCT recipients is a rare event that should be suspected in patients with persistent fever, pancytopenia and possible exposure to Leishmania spp., remembering that – as well as South-East Asia, East Africa and South America - it is endemic in several European regions.