Successful Treatment of Hypertrophic Pachymeningitis Complicated with Myelodysplastic Syndromes with 5-Azacytidine
Journal Title: Biomedical Journal of Scientific & Technical Research (BJSTR) - Year 2017, Vol 10, Issue 3
Abstract
It is known that the causative diseases of secondary hypertrophic pachymeningitis (HP) include infections, malignant tumors, autoimmune diseases. Although myelodysplastic syndromes (MDS) are often accompanied by certain autoimmune diseases, secondary HP with MDS is rare. We here describe the patient with HP complicated by MDS and have severe resistance to the steroid therapy and successfully treated with the combination of corticosteroids and 5-Azacytidine (5-Aza). Administration of 5-Aza might be a useful therapeutic option for patients with refractory autoimmune diseases complicated with MDS. Hypertrophic pachymeningitis (HP) are secondarily caused by various diseases such as infections, malignant tumors, and autoimmune diseases [1]. Myelodysplastic syndromes (MDS) are hematopoietic malignancies characterized by pancytopenia in the peripheral blood and is often complicated by autoimmune diseases [2-4]. However, there are few reports of MDS complicated by HP 5-Azacytidine (5-Aza) is the first-line treatment options for high-risk MDS patients, for whom allogeneic hematopoietic transplantation is not eligible due to the old age and/or comorbidities. Here we report the first case of MDS complicated by HP, which was successfully treated with the combination of 5-Aza and corticosteroids. Case Report A 75-year-old man was referred to our hospital due to recurrent fever and swelling of his palpebra for three months. On the first visit at our hospital, the left eye was protruded and there was no sign of meningeal irritation or obvious neurological abnormality. Plain CT scans of the head demonstrated the existence of sinusitis, otitis media. The laboratory examination revealed that serum C-reactive protein (CRP) level was elevated and serological test was negative for antinuclear antibodies, PR3-anti-neutrophil cytoplasmic antibody (PR3-ANCA) and MPO-anti-neutrophil cytoplasmic antibody (MPO-ANCA). Although the protein level increased to 116 mg/dl in the cerebrospinal fluid, the glucose level was normal and there was no increase in cell number. Despite the treatment with antibiotics for orbital cellulitis, high fever persisted for more than 10 days. Contrast enhanced magnetic resonance imaging (CE-MRI) of the brain showed the enhancement of bilateral dura mater (Figure 1), thus we diagnosed him as having HP In response to oral administration of prednisolone 40 mg/day, both fever and swelling of the palpebra were gradually dissolved. Also, the enhancement of the dura mater was found to disappear by CE-MRI (data not shown). Because of anemia and thrombocytopenia on admission, we performed bone marrow analysis. The bone marrow aspirate revealed 7.3% of myeloblasts and morphologic abnormalities indicative of MDS. Karyotypic analysis of the bone marrow showed many abnormalities and WT1 mRNA level also increased to 14 x103 copy/^L. From these findings, we diagnosed this patient as having MDS (RAEB-1, IPSS Int-2) complicated with HP. When the dose of prednisolone was tapered to 30 mg/day, fever and swelling of the palpebra recurred and repeated brain CE-MRI demonstrated the enhancement of bilateral dura mater (Figure 2). So, intravenous methylprednisolone pulse therapy (500 mg/day for 3 days) was given, followed by oral prednisolone 40 mg/day being tapered by 10 mg every 2 weeks. CRP level decreased and relapsed soon after and fever persisted. Because we considered a possibility that background MDS might influence HP, we administered azacytidine (5-Aza) (110 mg/body for 5 days with 4-week interval) for MDS. This combinational treatment led to a dramatic improvement of the clinical symptoms and laboratory data during the next few weeks. WT1 mRNA expression was decreased to the normal range after the second course of 5-Aza (Figure 3). Then, the dose of prednisolone was gradually tapered to 17.5 mg/day, and he was discharged from our hospital without recurrence.
Authors and Affiliations
Chisato Ashida, Yuji Nozaki, Daisuke Tomita, Toshihiko Shiga, Masanori Funauchi, Itaru Ma
Keywords
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- EP ID EP587333
- DOI 10.26717/BJSTR.2018.10.001932
- Views 141
- Downloads 0
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