Human T-cell lymphotropic virus (HTLV) is a human oncoretrovirus known to cause adult T-cell leukaemia/lymphoma (ATLL). Coinfection of human T-lymphotropic virus type 1 with Epstein-Barr virus (EBV) results in enhanced expression of the HTLV virus and leads to aggressive organ involvement from T-cell malignancy. It has also been observed that the prevalence of hepatitis B infection has been higher in patients with HTLV ATLL as compared with the general population in certain countries. We describe a case of a 34-year-old man who initially presented with leucocytosis, fatigue and conjunctival erythema. His radiological images revealed significant generalised adenopathy, and his flow cytometry analysis came back positive for CD4-positive T-cell lymphoma. He was subsequently diagnosed with HTLV-positive ATLL. Ultimately the patient was also diagnosed with acute hepatitis B and EBV. We describe a unique case of ATLL with coinfection with two other viruses, the association of which can be of potential prognostic value in guiding the treatment strategies for ATLL.
Orbital involvement with histologic necrosis is a rare manifestation of systemic sarcoidosis. The authors present a case of necrotizing dacryoadenitis in addition to non-necrotizing granulomas in a hypertrophic scar that is consistent with a diagnosis of sarcoidosis. A 60-year-old female presented with 2 months of painless right upper eyelid fullness and ptosis. CT imaging demonstrated right greater than left lacrimal gland enlargement. A biopsy demonstrated necrotizing granulomatous inflammation of the lacrimal gland. Additional workup was negative for infectious or lymphoproliferative disease. On further investigation, the patient noted thickening of a longstanding abdominal scar, and a subsequent punch biopsy of the scar demonstrated non-necrotizing granulomas suggestive of scar sarcoidosis. CT chest identified mediastinal lymphadenopathy. A diagnosis of sarcoidosis was determined. The authors thereby present an unusual case of 2 histologic variants of sarcoidosis presenting with necrotizing granulomatous dacryoadenitis and non-necrotizing scar granulomas.
The presentation of two 19 year old males with stage I non-Hodgkin lymphoma in the proximal tibia prompted an extensive review of institutional and national databases to assess if there is any statistical evidence that these reflected a previously overlooked syndromic pattern of presentation. The institutional records of a single institution were reviewed for presentation of non-Hodgkin lymphoma in bone. The records of two additional institutions were reviewed for all reports of non-Hodgkin lymphoma in the tibia. Analysis was performed on data from SEER (Surveillance, Epidemiology, and End Results) dichotomized to bone presentation in the lower extremity versus other bones. Institutional databases included 20 patients with tibial presentation of lymphoma with a median age of 22.5 years (versus 42 for all bone lymphomas; p<0.001). 18/20 were diffuse large B cell lymphoma, and all patients ≤ 40 achieved remission and apparent cure. Distinctive and unusual features were a tendency for bilateral involvement of the tibia and sclerotic changes on X-ray. SEER data included 808 cases of bone lymphoma; the fraction of cases presenting in the lower extremity vs other bone sites is higher at ages ≤ 40 years (38% vs 19%; p < 0.0001). Presentation in the lower extremity, as compared to other bone sites, confers 97% overall survival in patients ≤ 40 (vs. 82%; p = 0.01). This survival effect was independent of stage. In contrast, no significant difference in overall survival was identified for lower extremity versus non-lower extremity site for age > 40. These data show a previously undescribed syndromic pattern of disease presentation: bone lymphoma in young patients is likely to present in the lower extremity, specifically the proximal tibia, has atypical sclerotic features on x-ray, is often bilateral, and has an excellent prognosis compared to bone lymphomas at other sites matched for stage and age.
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