Determining residual adipose tissue characteristics with MRI in patients with various subtypes of lipodystrophy.

PURPOSE: We aimed to investigate residual adipose tissue with whole-body magnetic resonance imaging to differentiate between subtypes of lipodystrophy. METHODS: A total of 32 patients (12 with congenital generalized lipodystrophy [CGL], 1 with acquired generalized lipodystrophy [AGL], 12 with familial partial lipodystrophy [FPLD], and 7 with acquired partial lipodystrophy [APL]) were included. RESULTS: Despite generalized loss of metabolically active adipose tissue, patients with CGL1 caused by AGPAT2 mutations had a significant amount of residual adipose tissue in the scalp, earlobes, retro-orbital region, and palms and soles. No residual adipose tissue was noted particularly in the head and neck, palms and soles in CGL2 caused by BSCL2 mutations. CGL4 caused by mutations in the PTRF gene was characterized with well-preserved retro-orbital and bone marrow fat in the absence of any visible residual adipose tissue in other areas. No residual adipose tissue was observed in AGL. Despite loss of subcutaneous fat, periarticular adipose tissue was preserved in the lower limbs of patients with FPLD. Retro-orbital adipose tissue was surprisingly preserved in APL, although they lacked head and neck fat. CONCLUSION: Lipodystrophies are a heterogeneous group of disorders characterized by generalized or partial loss of adipose tissue, which can be congenital or acquired. Our results suggest that residual adipose tissue characteristics can help distinguish different subtypes of lipodystrophy.

A case of inflammatory pseudotumor in the spleen.

A case of an inflammatory pseudotumor of the spleen is presented. A 43-year-old woman was hospitalized for a detailed examination due to pancytopenia, which was diagnosed during an examination related to left upper quadrant abdominal pain. Physical examination revealed a 2 to 3 cm splenomegaly. Reticulocyte count was 4% and erythrocyte sedimentation rate was 55 mm/h. No other important findings were noted, except for an evident increase in myeloid series and megakaryocytes (more evident in erythroid series) on bone marrow aspiration and biopsy. Computed tomography (CT) scan revealed splenomegaly and a centrally located hypodense lesion with a 92 X 86 mm axial diameter in the spleen. With a hematologic malignancy prediagnosis, a splenectomy was performed on the patient because of pancytopenia and splenomegaly. An intrasplenic, centrally located, well-limited, capsulated lesion (9.5 x 11 x 10 cm in diameter) was discovered on macroscopic examination of the material. A cellular infiltration area was seen on microscopic examination. The spleen capsule was mildly fibrotically thickened. The lesion that separated from the spleen tissue consisted of diffusely proliferated fusiform fibroblasts, heterogenous inflammatory cells consisting mainly of plasma cells, lymphocytes, sparse neutrophils and vascular elements. No granuloma or multinuclear giant cells were detected. Pancytopenia improved on follow-up. The patient followed up for two years, is now healthy and has no complaints.