Abstract | November 8, 2021

Disseminated Histoplasmosis associated Hemophagocytic Lymphohistiocytosis (HLH) in an Immunosuppressed Patient

Presenting Author: Azad Patel, MD, Internal Medicine Resident PGY2, Department of Medicine, Johnston Memorial Hospital, Abingdon, VA, Abingdon, VA

Coauthors: Nirav Patel, MD, Internal Medicine, PGY3, Johnston Memorial Hospital, Abingdon, VA, Jaimin Kapadia, Medical student, LMU, TN, Christopher Morris, MD, Rheumatologist, Kingsport, TN, Michael S. Stump, MD, Pathologist, Carilion Clinic, Roanoke, VA, Gail Stanley, MD, Infectious disease, Johnston Memorial hospital, Abingdon, VA, Camelia Chirculescu, MD, Program Director, Internal Medicine, Johnston Memorial Hospital, Ballad Health, Abingdon, VA.

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Introduction: HLH is a life-threatening rare disorder which is triggered by infection and alteration of immune-homeostasis in adults. Here we report a case of HLH occurring as complication of disseminated histoplasmosis in a patient with sarcoidosis treated with Adalimumab and Methotrexate. 

Case Presentation: A 62-year-old woman with h/o hypertension, diabetes-mellitus, chorioretinitis, recurrent uveitis secondary to sarcoidosis on Adalimumab, Methotrexate presented to ED with encephalopathy. On admission, patient’s Labs were remarkable for sodium 130, AST 208, ALT 65, Ammonia 43, Albumin 2.2, WBC 4.7 with bandemia, HGB 10.6, and platelets 88K, CRP 207.8, ESR 62. CT head, chest x-ray, urinalysis, were unrevealing. CT abdomen showed splenomegaly, portal-lymphadenopathy. Treatment for multifactorial encephalopathy was initiated with IV fluids, Piperacillin/Tazobactam, Vancomycin. On 3rd-day patient had fever of 102F with pancytopenia. WBC decreased from 4.2-to-2.4, HGB 10.6-to-9.7, Platelets 88k-to-48k, D-dimer>5000, Fibrinogen 167, ferritin>1500, Triglyceride 218. Hepatitis-panel, HIV-panel, Blood cultures, Peripheral-smear, flow-cytometry, ANA, Rheumatoid-factor, influenza, COVID-19 were negative. Infectious Disease recommends antibiotic-changes from Piperacillin/Tazobactam to Cefepime due to neutropenia. Oncologist recommended a bone marrow biopsy for further evaluation of pancytopenia. On 5th-day urine histoplasmosis-Ag was positive. 

Diagnosis: The patient was determined to meet 5 out of 8 criteria for HLH syndrome initially: 1)Fever, 2)Splenomegaly, 3)Cytopenia>2 cell-lines, 4)Hypertriglyceridemia or Hypofibrinogenemia(<150), 5)Hyperferritinemia(>500). 

Management: Treatment with Itraconazole, IV Methylprednisolone 1gm/daily for 3-days was started on recommendation of rheumatologist. Treatment led to improve patient’s mentation. Patient became afebrile and was transferred to tertiary-center for further treatment. Bone-marrow biopsy identified hemophagocytosis and yeast forms consistent with Histoplasma morphology. It also indicated 98% H-score on calculation confirming the diagnosis of disseminated histoplasmosis and HLH. Patient was treated with IV-liposomal Amphotericin-B for 4-weeks followed by oral Itraconazole. 

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