Hepatosplenic T-cell lymphoma (HSTCL) is an extremely rare and aggressive form of non-Hodgkin lymphoma associated with poor response to treatment and high mortality. There is an increased incidence among patients with inflammatory bowel disease, especially young male patients under 35 years old and on combination therapy (thiopurine and anti-TNF-α). We describe a case of HSTCL in a young male patient with stenosing ileal Crohn’s disease on azathioprine monotherapy for 4.8 years admitted to our hospital with intra- abdominal sepsis. Despite chemotherapy, the patient eventually died 1 month after the diagnosis. Through a literature review, we identified 18 additional cases of HSTCL in Crohn’s disease patients that had only been treated with thiopurine monotherapy. The authors intend to highlight the rarity of this diagnosis especially with azathioprine monotherapy and the diagnostic challenge in a case that presented with intra-abdominal sepsis.

his legs. His primary laboratory investigation showed anemia, monocytosis, eosinophilia and thrombocytopenia,. He also had elevated serum creatinine (4.4 mg/dl) and LDH(430U/L) values respectively. His prior creatinine level was normal. His urine analysis showed 28 erythrocytes/ hpf. Calculated proteinuria was 750 mg/day. Urinary ultrasound examination was normal. Protein electrophoresis showed hyper gamma globulinemia 1.73 g/dl. Leucocytoclastic vasculitis was revealed by punch biopsy of skin. All serologic workup was normal except for low C3 levels (77 mg/dl). Bone marrow biopsy and renal fine needle biopsy was performed. Bone marrow biopsy showed peritrabecular and intertrabecular lymphoid nodules consisting of CD20, CD5, Bcl-1 positive atypical lymphoid cells regarded as neoplastic infiltration. Renal biopsy showed intravascular and interstitial atypical lymphoid cells as groups of 4-5 cells. These atypical lymphoid cells were CD20, CD5 Pax-5 and Bcl-1 positive. Pathological and immune-histochemical findings of biopsies were consistent with MCL. Positron emission tomography determined iliac, obturatory lymph nodes and avidity at renal hilum level. Patient was planned to receive 6 cures of Rituximab, cyclosporine, hydroxydaunorubicine, oncovine, prednisone. After first R-CHOP cure he suffered a massive pulmonary emboli and died. Conclusions: Infiltration of renal parenchyma by lymphoma cells is very rare and is rated in about 1 % of cases. There are only three cases of renal MCL infiltration with ARF. Our case is the fourth case of MCL presenting with ARF due to neoplastic cell infiltration while this is the first case that neoplastic MCL cells to be shown in the lumen of renal vessels, capillaries. The case demonstrates for the first time the possibility of intravascular renal infiltration by MCL. It also revealed the importance of the renal biopsy as a useful diagnostic choice in case of kidney impairment in lymphoma patients.

Introduction: Kikuchi-Fujimoto disease (KFD) is a rare, benign, necrotizing lymphadenitis of unknown aetiology with good prognosis. It is characterized by cervical lymphadenopathy, nocturnal diaphoresis and fever. Surgical excision of the adenopathy, histopathological study and immunophenotyping are crucial for diagnosis. Patients and methods: This paper describes five patients with three different histological subtypes of KFD, including an atypical presentation masquerading as pyelonephritis and two other cases where physicians mistakenly started chemotherapy. In one other case cytomegalovirus was identified as the responsible aetiological agent, while in the remaining patient, KFD evolved into an autoimmune condition. Discussion: KFD, although rare, may mimic infectious, autoimmune and neoplastic diseases. It also poses a risk for the subsequent development of an autoimmune disorder.