Acquired Hemophagocytic Lymphohistiocytosis : It’s Not Just in the Genes

1University Hospitals Case Western Reserve/Rainbow Babies and Children's Hospital, Cleveland, OH
2University Hospitals Case Western Reserve, Cleveland, OH

Meeting: Hospital Medicine 2015, March 29-April 1, National Harbor, Md.

Abstract number: 649

Keywords:

Case Presentation:

A 71 year old woman with a history of transformed diffuse large B-cell Non-Hodgkin’s Lymphoma, currently in remission, was transferred to our hospital for several days of malaise and persistent fevers. She was initially treated at an outside hospital where she received multiple antibiotic regimens, and had a negative systemic workup. On transfer, she displayed altered mentation, new hypoxia, high fevers, tachypnea, and tachycardia with preserved blood pressure. Laboratory evaluation revealed mildly elevated lactate and troponin levels (both subsequently resolved) and anemia. White blood cell count was not elevated, and differential was within normal limits. An ECG showed sinus tachycardia, and her initial arterial blood gas was indicative of respiratory alkalosis. Chest x-ray showed small bibasilar pleural effusions with mild alveolar infiltrates consistent with edema. 

On transfer she was treated with broad spectrum antibiotics/antifungals. However, she developed hypoxic respiratory failure with altered mental status resulting in ICU transfer and intubation. MRI of her head was normal. All cultures (urine, blood, CSF) were negative. CT of her chest and spine revealed a concerning lesion in the lower thoracic spine. Biopsy revealed fibrotic material. A peripheral smear showed toxic granulation consistent with an infectious source. During her inpatient course the patient developed elevated transaminases with normal bilirubin, neutropenia, thrombocytopenia, and persistent anemia. A PET scan showed several hypermetabolic foci along the lower thoracic spine, consistent with previously biopsied fibrous tissue. Bone marrow biopsy showed atypical cellular infiltrates suspicious for large cell lymphoma or Hodgkin’s Lymphoma. B-cell stains were negative, with multiple CD30+ cells present. Additionally, there was prominent hemophagocytosis with negative AFB, GMS, and ISH for EBER stains. Depressed fibrinogen, and elevated triglycerides and ferritin >11000 were then noted, and she was diagnosed with Hemophagocytic Lymphohistiocytosis (HLH). Treatment concordant with the HLH 2004 guidelines (etoposide, dexamethasone) was initiated, and her condition slowly improved.

Discussion:

HLH is a syndrome which may be genetic, or acquired, and related to diverse etiologies. The presenting disease process is characterized by a nonspecific constellation of fevers, cytopenias, and hepatosplenomegaly. When seen in complex patients such as ours, the vague presentation of such a potentially life threatening disorder can prove to be a diagnostic challenge. Laboratory information can be of significant assistance, displaying more unique attributes of HLH including hyperferritinemia, hypertriglyceridemia, hypofibrinogenemia, and transaminitis. Inciting events have been described including acute/chronic infectious sources, rheumatologic diseases, immunosuppression, and various hematologic/oncologic diagnoses. In our patient, the origin of HLH was thought to be secondary to lymphoma, however subsequent testing did not show definitive proof of relapse.

Conclusions:

Although relatively rare in adult patients, acquired HLH follows a similar pattern of symptoms as its genetic counterpart seen in children. The hallmarks of physical diagnosis in HLH include cytopenia, organomegaly, and high unexplained fevers. Considering HLH as part of a differential diagnosis in adults is important when encountering similar findings in hospitalist medicine.

To cite this abstract:

Peluso C, Shaniuk P, Stehouwer N, Armitage K, Strausbaugh S. Acquired Hemophagocytic Lymphohistiocytosis : It’s Not Just in the Genes. Abstract published at Hospital Medicine 2015, March 29-April 1, National Harbor, Md. Abstract 649. Journal of Hospital Medicine. 2015; 10 (suppl 2). https://www.shmabstracts.com/abstract/acquired-hemophagocytic-lymphohistiocytosis-its-not-just-in-the-genes/. Accessed November 12, 2019.

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