Homophagocytic Syndrome: A Case Report and Literature Review

1Johns Hopkins University School of Medicine, Baltimore, MD
2Johns Hopkins University School of Medicine, Baltimore, MD
3Johns Hopkins University School of Medicine, Baltimore, MD

Meeting: Hospital Medicine 2010, April 8-11, Washington, D.C.

Abstract number: 358

Case Presentation:

This is a 24‐year‐old man of Filipino descent with no significant past medical history who presented with a 10‐day history of fever, chills, anorexia, and fatigue. He decided to seek medical attention after experiencing 2 syncopal events within 24 hours. The day before admission he passed out while in the shower when getting up from a squatting position, and on the day of admission he passed out at work while getting up from a seated position. He went to an urgent care center and was found to be febrile and orthostatic. Initial labs revealed he had acute renal failure (Cr 3.2 mg/dL), hepalic dysfunction (ALT of 427, AST of 556), and pancytopenia (WBC 2.2 Hb 10.9, Plt 53). He was subsequently directly admitted to the hospital. As part of his evaluation for anemia, a ferritin level was drawn and was markedly elevated at 15,289 ng/mL The patient was suspected to have he mo phagocytic syndrome because of the constellation of fever, neutropenia, thrombocylopenia, and elevated ferritin (> 500 ng/mL). The patient was evaluated for the most common causes of hemophagocylic syndrome including Epslein‐Barr Virus (EBV), cytomegalovirus (CMV), HIV. parvovirus, lupus, rheumatoid arthritis, and Sjögren syndrome, none of which were present. An initial bone marrow biopsy performed on hospital day 4 was nondiagnostic, but the patient was started on empiric intravenous steroid therapy because of the high clinical suspicion for hemophagocylic syndrome. With this treatment he defervesced and his leukopenia, thrombocytopenia, hepatic dysfunction and renal failure resolved. On hospital day number 11 a repeat bone marrow biopsy showed hemophagocylosis, confirming the diagnosis.

Discussion:

Hemophagocytic syndrome is a very rare condition in adults, with only 116 cases reported in the literature since 1983. It is caused by a dysfunction in natural killer T‐cell function causing uncontrolled hemophagocylosis by lymphocytes and histiocytes. The syndrome is characterized by fever, hepatosplenomegally, cytopenias, liver dysfunction, and elevated levels of ferritin. It is usually divided into 2 categories, a primary or genetic hemophagocytic syndrome and a secondary or reactive type. The primary form is seen most commonly in infants and young children and has a mortality rate of 45%. Secondary hemophagocytic syndrome has a better prognosis and has been associated with rheumatologic diseases (lupus, rheumatoid arthritis, Sjögren syndrome, sarcoidosis, and polyarteritis nodosa), viral infections (CMV, EBV, HIV, VZV. and HHV‐8), and rarely with bacterial and parasitic infections. In adults there have been so few cases thai there are no guidelines for treatment. Our patient did well because he presented and was treated prior to permanent multiorgan system failure.

Conclusions:

The purpose of this case report was to highlight this rare but potentially fatal condition.

Author Disclosure:

A. Sridharan, none; D. Chang, none; C. Hesdorffer, none.

To cite this abstract:

Sridharan A, Chang D, Hesdorffer C. Homophagocytic Syndrome: A Case Report and Literature Review. Abstract published at Hospital Medicine 2010, April 8-11, Washington, D.C. Abstract 358. Journal of Hospital Medicine. 2010; 5 (suppl 1). https://www.shmabstracts.com/abstract/homophagocytic-syndrome-a-case-report-and-literature-review/. Accessed August 24, 2019.

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