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Rare and Fatal Acquired Immunodeficiency Syndrome (AIDS) Related Malignancy Complicating Multisystem Organ Failure FREE TO VIEW

Michael Krol, MD; Abdulla Majid-Moosa, MD; Adan Mora, MD
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Baylor University Medicial Center, Dallas, TX

Chest. 2015;148(4_MeetingAbstracts):263A. doi:10.1378/chest.2250303
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SESSION TITLE: Critical Care Student/Resident Case Report Posters I

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: AIDS related malignancies may alter clinical courses and result in death in critically ill patients. We present a case of a newly diagnosed AIDS patient with Pneumocystis jiroveci pneumonia (PJP), Epstein-Barr Virus, and Cytomegalovirus (CMV) infections found to have anaplastic lymphoma kinase (ALK) negative Anaplastic Large Cell Lymphoma (ALCL).

CASE PRESENTATION: A 55 year-old-man newly diagnosed with AIDS presented with fever, dyspnea, non-productive cough, and weight loss. He only had two days of highly active antiretroviral therapy. On exam, he was tachypneic and had thrush. He was pancytopenic (white blood cell 4.3K/uL, hemoglobin 8.7 g/dL, platelets 124 K/uL), had a lactate of 4.3 mg/dL, elevated lactate dehydrogenase (LDH) of 900 IU/L, and PaO2 was 40 mm Hg. His CD4 cell count was 5/mm3 and viral load was 609,639 copies/ml. He was treated for PJP with steroids, bactrim and then pentamidine after limited clinical improvement, persistent elevation in LDH, bandemia, and lactic acidosis. Valganciclovir was started for a positive CMV PCR. On hospital day 10 he required intubation and had a new finding of enlarged inguinal lymph nodes. He developed worsening multiorgan failure and died on hospital day 14. An Autopsy was performed. Autopsy was notable for splenomegaly, hepatomegaly, and widespread lymphadenopathy. Neoplastic lymphoid cells were diffusely immunohistochemically positive for CD30, focally positive for CD3 (a T-cell marker) and epithelial membrane antigen. Cells were strongly positive by Epstein-Barr virus encoded RNA and were negative for ALK protein. Mitotic activity, or Ki-67, measured 20-25% with focal areas of 60-65%. The final diagnosis was advanced metastatic ALK-negative ALCL arising in a setting of AIDS.

DISCUSSION: Peripheral T Cell Lymphoma’s (PTCL) arise from mature T cells. PTCL account for 5-10% of Non Hodgkin lymphoma’s with an incidence <1 per 100,000 patients. PTCL is subdivided into nodal, extranodal and leukemic subgroup. Within the nodal group, ALCL is the least common subtype representing 12% of the PTCL cases worldwide. ALK negative ALCL is more aggressive and less responsive with studies suggesting 5 year overall survival rates of 49% vs.70% for ALK positive ALCLs. ALCL involvement in HIV patient's is limited to case reports, with only 11 of 37 patients identified in the largest case review showing AIDS defining illness on presentation. Lymphomas are known to cause persistent lactic acidosis and cause an overwhelming tumor burden which can obscure the clinical picture.

CONCLUSIONS: Critically ill AIDS patients may appear refractory to conventional therapies in the setting of unsuspecting rare malignancies. They require a broader differential diagnostic approach and high index of suspicion for occult malignancies

Reference #1: Perez K, Castillo J, Dezube BJ, Pantanowitz L. Human Immunodeficiency Virus-associated Anaplastic Large Cell Lymphoma. Leukemia & Lymphoma 2010; 51(3): 430-38.

DISCLOSURE: The following authors have nothing to disclose: Michael Krol, Abdulla Majid-Moosa, Adan Mora

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