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

Immune System Gone Awry: A Story of Lost Opportunity FREE TO VIEW

Robert Brammer, MD; Kumar Vipul, MD
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Wellspan York Hospital, York, PA

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

Chest. 2016;150(4_S):1082A. doi:10.1016/j.chest.2016.08.1189
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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: Macrophage activation syndrome (MAS) is a rapidly progressing syndrome of hyper-active immune system. It is a form of hemophagocytic lymphohistiocytosis (HLH) occurring in patients with rheumatologic diseases typically seen in infants and young adults.

CASE PRESENTATION: An 80-year old male with chronic rheumatoid arthritis treated with prednisone and hydroxychloroquine was hospitalized due to worsening dyspnea and 20 pound- weight loss in 2 months. CT revealed multi-focal ground glass infiltrates, mediastinal adenopathy, and splenomegaly (20cm.). His bronchoscopy was unrevealing and was treated for community-acquired pneumonia. He had pancytopenia with WBC count 1.8 K/mcl and platelets 47 K/mcl. During outpatient bone marrow biopsy 4 weeks later, he was found to be hypotensive, forgetful, anorexic and diaphoretic. He was transferred to the intensive care unit. Physical examination showed hepatomegaly, splenomegaly, axillary lymphadenopathy and hypotension. Since clinical suspicion for MAS was high, appropriate laboratory tests were ordered and he was started on dexamethasone 10 mg every 8 hours. Appropriate blood products were transfused. He deteriorated rapidly requiring several vasopressors and family decided to withdraw care on day 5. Our patient met 5/8 diagnostic criteria used in HLH-2004 trial: Splenomegaly, peripheral blood cytopenia (WBC - 1.3 K/mcl, Hemoglobin 7.0 gm/dL, and platelets 19 K/mcl), Hypofibrinogenemia - 92 mg/dL, Macrophages containing cellular material in the bone marrow and Ferritin level > 7500 ng/ mL. We believe his initial presentation was related to MAS and our case highlights the difficulty in diagnosing this syndrome.

DISCUSSION: HLH can present as a single occurrence or as relapsing occurrences. The initial trigger for an acute attack in either an infection or a change in immune balance. In MAS, there is immune deficiency in combination with heightened inflammation. It is imperative to rule out infection in such a situation. Our patient was found to have scattered non-necrotizing granulomas in the bone marrow. The fungal and acid-fast stains were negative. Since culture results were not available, there was hesitancy to start HLH specific chemotherapeutic agents.

CONCLUSIONS: MAS can present in older patients at any time during the course of rheumatologic disease. Whether it is a presenting manifestation, during relapses or during a concurrent infection, diagnosis remains challenging.

Reference #1: Clinical characteristics, prognostic factors, and outcomes of adult patients with hemophagocytic lymphohistiocytosis. Otrock ZK, Eby CS. Am J Hematol. 2015 Mar;90(3):220-224.

Reference #2: Reactive hemophagocytic syndrome in adult systemic disease: report of twenty-six cases and literature review. Dhote R,, Christoforov B et al. Arthritis Rheum. 2003;49(5):633.

Reference #3: Prognostic factors and outcomes of adults with hemophagocytic lymphohistiocytosis. Parikh SA, Kapoor P, Letendre L, Kumar S, Wolanskyj AP. Mayo Clin Proc. 2014;89(4):484.

DISCLOSURE: The following authors have nothing to disclose: Robert Brammer, Kumar Vipul

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