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Hyperlipidemia, Carotenoderma, and Respiratory Failure: An Unusual Initial Presentation of Multiple Myeloma FREE TO VIEW

Nicole Carreau, MD; Natanyah Siegel, MD; Shira Saul, MD; Janice Gabrilove, MD; Kusum Mathews, MD
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Mount Sinai Hospital, New York, NY

Chest. 2015;148(4_MeetingAbstracts):882A. doi:10.1378/chest.2242544
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SESSION TITLE: Pulmonary Manifestations of Systemic Disease Student/Resident Case Report Posters II

SESSION TYPE: Student/Resident Case Report Poster

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

INTRODUCTION: Multiple myeloma (MM) classically presents with hypercalcemia and renal failure. Clinical course may be complicated by respiratory failure, but it is rarely observed prior to diagnosis. We present a case where an uncommon MM variant presented as acute multi-organ failure.

CASE PRESENTATION: A previously healthy, 57 year old man presented to his primary doctor with worsening orange skin discoloration. Labs revealed hypertriglyceridemia (3124mg/dl) and transaminitis. Outpatient imaging showed left lower lung consolidation and hepatic fibrosis. Serum IgG rose from 4600 to 5643 mg/dl over two weeks. Within 30 days, he was hospitalized for fever, acute liver failure, diffuse colitis, and acute kidney injury requiring hemodialysis. He rapidly decompensated, requiring intubation for hypoxemic respiratory failure. Repeat imaging showed bilateral, multi-lobar consolidations and ground glass opacities concerning for acute respiratory distress syndrome. Bronchoalveolar lavage viral culture was positive for Influenza A. Extensive testing was notable for mildly elevated serum beta carotene (205ug/dl), high apolipoprotein B (230mg/dl), and paraproteinemia. Skin biopsy showed xanthoma. Immunofixation and bone marrow biopsy confirmed a new diagnosis of IgG-lambda MM. After 48 hours he was successfully extubated, but remained hypoxemic with high supplemental oxygen requirement for several days. Dyslipidemia and renal failure improved with chemotherapy, and he was discharged with close follow-up.

DISCUSSION: Hyperlipidemic myeloma (HLM), more commonly seen with the IgA subtype, accounts for 6% of MM cases. Typically diagnosis is delayed, with xanthomas or hyperlipidemia preceding other symptoms by years. Patients have been described as having “orange skin,” or carotenoderma. The underlying mechanism of the hyperlipidemia remains unclear. Respiratory failure is uncommon at presentation, but one-third of MM patients later develop pulmonary infectious complications, due to immunocompromised status. This patient’s underlying MM likely predisposed him to multi-organ failure and respiratory failure triggered by influenza, with lab abnormalities prompting workup for malignancy.

CONCLUSIONS: MM uncommonly presents with acute multi-organ failure, including respiratory failure. Hyperlipidemia, xanthomas, or carotenoderma may portend development of the rarer variant, HLM. Hematologic malignancies should be considered in the differential for patients with multi-system dysfunction, not otherwise explained.

Reference #1: Misselwitz B, Goede JS, Pestalozzi BC, Chanz U, Seebach JD. Hyperlipidemic myeloma: review of 53 cases. Ann Hematol 2010;89:569-77.

Reference #2: Poe RH, Kamath C, Bauer MA, et al. Acute respiratory distress syndrome with pulmonary calcification in two patients with B cell malignancies. Respiration. 1989; 56:127-33.

Reference #3: Kintzer JS, Rosenow EC, Kyle RA. Thoracic and Pulmonary Abnormalities in Multiple Myeloma: A Review of 958 Cases. Arch Intern Med. 1978;138(5):727-30.

DISCLOSURE: The following authors have nothing to disclose: Nicole Carreau, Natanyah Siegel, Shira Saul, Janice Gabrilove, Kusum Mathews

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