GROSS HEPATOSPLENOMEGALY IN A PATIENT WITH HUMAN HERPES VIRUS-8 ASSOCIATED MULTICENTRIC CASTLEMAN'S DISEASE
Abstract
A 30 year old man presented with a three week history of worsening dyspnoea, productive cough and fever. Examination revealed cervical lymphadenopathy, splenomegaly and bilateral cutaneous lesions on the legs. The patient denied any medical co-morbidities or any recent travel. Blood tests showed anaemia (haemoglobin of 76 g/L), thrombocytopenia (45×103/mm3) a white blood cell count of 5.1×103/mm3 and a CRP of 61 mg/dL. A HIV test was positive with a CD4 count of 12 count cells/mm3 and a HIV Viral Load of 1420045 copies/ml.
A punch biopsy was taken from the leg lesions was positive for HHV-8 in keeping with the diagnosis of Kaposi Sarcoma. Computed tomography (CT) scan of the thorax and abdomen (Figure-1) demonstrated mediastinal lymphadenopathy, lungs nodularity and bilateral patchy opacities, and bronchoscopy revealed Kaposi sarcoma lesions in the trachea and main lobar bronchi. A cervical lymph node showed marked plamacytic hyperplasia with focal EBV positivity and regression of germinal centers, in keeping with a diagnosis of HHV-8-associated multi-centric Castleman's disease. Investigations for atypical pneumonia, TB and PCP were all negative.
Despite the patient receiving antiretrovirals, antibiotics, steroids and blood transfusions the patient developed multi-organ failure and died shortly after.
Multi-centric Castleman's disease (MCD) is rare lymphoproliferative disorder, with 50% of these cases caused by HHV-8, which also causes Kaposi Sarcoma.1 It presents with generalized lymphadenopathy, hepatosplenomegaly, anaemia, hyper-immunoglobulinaemia, and systemic symptoms such as fevers, night sweats and weight loss.2
Diagnosis of Castleman's disease is based on lymph node biopsy which can demonstrate Hyaline-Vascular, plasmablastic and plasma Cell variants of Castleman's disease. (Figure-2)
References
Aoki Y, Yarchoan R, Wyvill K, Okamoto S-i, Little RF, Tosato G 2001. Detection of viral interleukin-6 in Kaposi sarcoma-associated herpesvirus-linked disorders. Blood 97:2173-6.
Zhao X, Shi R, Jin X, Zheng J. Diffuse hyperpigmented plaques as cutaneous manifestation of multicentric Castleman disease and treatment with thalidomide: Report of three cases. J Am Acad Dermatol 2011;65:430-2.
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