Pulmonary Manifestations of Systemic Disease: Student/Resident Case Report Poster - Pulmonary Manifestations of Systemic Disease I |

The Maze in the Castle: The Challenging Diagnosis of a Rare Cause of Diffuse Lymphadenopathy in HIV/AIDS FREE TO VIEW

David Gerling, MD; Harish Seethapathy, MD; Joseph Gabriel, MD
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Rochester Regional Health, Rochester, NY

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):1083A. doi:10.1016/j.chest.2016.08.1190
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SESSION TITLE: Student/Resident Case Report Poster - Pulmonary Manifestations of Systemic Disease I

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Castleman’s disease is a rare lymphoproliferative disorder associated with Human Immunodeficiency Virus (HIV) and Human Herpesvirus 8 (HHV-8). It can be unicentric or multicentric, with the latter portending a worse prognosis. HIV patients with rapidly progressing multicentric disease can die within weeks of diagnosis due to infection, disease progression, or associated malignancies. Definitive diagnosis is also often delayed due to the rarity of the disease and the vague, non-specific symptoms that manifest. Here we present an AIDS patient with multicentric Castleman’s disease.

CASE PRESENTATION: A 49 year old male with AIDS on antiretroviral therapy (CD4 113 cells/μL) presented with five days of progressively worsening diffuse abdominal pain, night sweats, generalized fatigue and malaise. Physical exam revealed enlarged and tender cervical, supraclavicular, axillary and inguinal lymph nodes. Labs revealed normocytic anemia and thrombocytopenia. Shortly after admission, he developed high-grade fevers (39-40 degree Celsius) and was started on broad-spectrum antibiotics. This was followed by a complete infectious workup. CT scan demonstrated hilar and mediastinal lymphadenopathy along with hepatosplenomegaly and diffuse lymphadenopathy throughout the abdomen and pelvis. An excisional supraclavicular node biopsy was performed on day three of hospitalization. Soon after, positive serologies for Bartonella Quintana (IgG 1:512, IgM negative) returned, and he was started on doxycycline for treatment of bacillary angiomatosis. Nine days into hospitalization, pathology revealed atypical interfollicular plasmacytosis with focal lamda light chain-restricted immunoblasts and HHV-8 positive cells, consistent with multicentric Castleman’s disease. Bartonella Quintana stains (Warthin-Starry and Bartonella immunohistochemistry) from the lymph node sample were negative. Doxycycline was discontinued and the patient was referred to oncology for follow-up with plans to start treatment with gancyclovir, rituximab and chemotherapy.

DISCUSSION: Multicentric Castleman’s disease is seen in HIV patients whose CD4 count is usually greater than 200 cells/μL and is associated with other malignancies such as Kaposi’s sarcoma and lymphoma. Risk factors include increasing age and no prior antiretroviral therapy. Notably however, our patient was on HAART and had a CD4 count less than 200 cells/μL at time of diagnosis. Patients often present with non-specific symptoms, of which fever and lymphadenopathy are the most common. The diagnosis is often delayed while patients are evaluated for more common etiologies. Definitive diagnosis requires biopsy and treatment varies depending on HHV-8 positivity. Typical regimens can include antiviral, immunotherapy and chemotherapy.

CONCLUSIONS: Our patient’s presentation highlights the difficulty in diagnosing this rare disease.

Reference #1: Casper C. The aetiology and management of Castleman disease at 50 years Br J Haematol 2005; 129:3

DISCLOSURE: The following authors have nothing to disclose: David Gerling, Harish Seethapathy, Joseph Gabriel

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