A case of Weber?Christian disease associated with myelodysplastic syndrome

Abstract

We report the case of a 73-year-old man with myelodysplastic syndrome (MDS) who developed Weber-Christian disease (WCD). Bone marrow aspirates showed refractory anemia with abnormal karyotypes such as trisomy 8, trisomy 8 and 14, and trisomy 8, 9, and 14. The patient had intermittent fever associated with multiple tender erythematous nodules on the skin. A biopsy sample taken from a nodule revealed focal subcutaneous infiltration of neutrophils and necrotizing fat tissue. We diagnosed the patient as having lobular panniculitis associated with myelodysplastic syndrome. The serum levels of soluble interleukin-2 (IL-2) receptor, interferon-Gamma, IL-1-Beta, IL-6 and tumor necrosis factor-Alpha were elevated in the active state but returned to normal after prednisolone therapy. This finding appears to implicate a T-cell immune response in the pathogenesis of Weber-Christian disease.

Subcutaneous Sweet syndrome with nuclear segmentation anomalies: a diagnostic marker of myelodysplasia

“…1 There have been nine reported cases of neutrophilic panniculitis associated with MDS (Table 1). [2][3][4][5][6][7][8][9] These nine patients had a mean age of 58 years with a male predominance. Most cases were accompanied by fever, with the limbs being the predominant site of lesion occurrence.…”

Pfeifer-Weber-Christian Disease: A Case Report and Review of Literature on Visceral Involvements and Treatment Choices

“…9,16,17 The findings of bone marrow aspirate varied from considerable retardation of the maturity of granulocytic series, 1 to refractory anemia, characterized by several erythroid cells with two nuclei, neutrophils with Pelger-Hüet-like nuclei or without granules, and megakaryocytes with multisegmental nuclei or multiple nuclei. 23 The myelodysplastic syndrome (MDS) in PWCD is rarely reported. 23,[33][34][35]…”

“…The clinical features of hematological involvement are anemia 1,5,19,23,32 (mostly hypochromic and microcytic, 3,14,24 occasionally normochromic and microcytic) 16 , leucopenia 3,6,20 (with mainly neutropenia), 19 and thrombocytopenia. 5,11 Pancytopenia is rarely described.…”