Coexisting Hemophagocytic Lymphohistiocytosis, Clostridium difficile Complicates Diagnosis

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Investigators emphasize the importance of a timely HLH diagnosis and prompt initiation of treatment, as well as close outpatient follow-up to increase patient survival.

Because of similar presentation, concurring diagnosis of hemophagocytic lymphohistiocytosis (HLH) and Clostridium difficile may not take place, which can contribute to the underdiagnosis of HLH, according to investigators of a case study published in Cureus.1

Investigators emphasized the importance of a timely HLH diagnosis and prompt initiation of treatment as well as close outpatient follow-up to increase patient survival.1

HLH is a rare immune disease where histiocytic and lymphocytic white blood cells attack other blood cells, causing the liver and spleen to enlarge, according to Johns Hopkins Medicine.2

In the case study, investigators followed a 29-year-old female patient who had a history of HLH, which presented with nausea, non-bloody diarrhea, and vomiting.1

Previously, the patient was treated with dexamethasone, etoposide, and intravenous immunoglobulin (IVIG). The patient was supposed to start intrathecal methotrexate, but she developed transaminitis with lower extremity pain and swelling.1

The patient was lost to follow up until presentation to the hospital part of this study. When at the hospital, she was febrile, hypotensive, and tachycardic.

She had ferritin levels of 13,858, investigators said.1

She was then evaluated for C diff because of being immunocompromised from chronic steroid use. The patient tested positive and was started on oral vancomycin.1

Investigators performed a computerized tomography scan of the abdomen and pelvis, which showed, edema, hepatosplenomegaly, pan-colonic bowel wall mural thickening, and small mesenteric and periportal lymph nodes.1

The patient later developed hemodynamic instability, and the antibiotics were switched to broad spectrum.1

Because of the patient’s previous HLH status and decompensated clinical status, the investigators started her on IVIG and steroids. She also required intubation and developed diffusely scattered petechiae.1

Investigators diagnosed her with severe anemia and thrombocytopenia that persisted, even with blood and platelet transfusions.

The patient continued to decompensate and eventually died.1

Investigators noted the challenges associated with identifying an HLH flare compared with sepsis secondary to an C diff infection.

Ferritin levels were checked 3 days into admission and were slightly elevated but were also persistent, they said.

There are limited data on ferritin trends related to relapsing or treated HLH, though it has been argued that it could be predictive of treatment outcomes and improved survival for those with HLH.1

The persistent elevated levels complicated the entire diagnostic picture of identifying the HLH flare, investigators said.1

Investigators said in their case, and 2 cases investigated, the patients quickly decompensated when there were coexisting diseases, but because of the similar presentation of HLH and septic presentation, further diagnosis is challenging.1

Close outpatient follow-up of the initial HLH diagnosis could have helped improve clinical outcomes in this case, investigators said.

The health disparity in this case may have contributed to the patient’s morbidity and mortality, given that she was not on the optimal therapy for the disease, investigators said.1

References

1. Boldig K, Kiamos A, Agrawal A, Reddy P. Hemophagocytic lymphohistiocytosis and Clostridium difficile infection: a diagnostic dilemma. Cureus. 15(1):e33865. doi:10.7759/cureus.33865

2. Hemophagocytic lymphohistiocytosis. Johns Hopkins Medicine. Accessed January 27, 2023. https://www.hopkinsmedicine.org/health/conditions-and-diseases/hemophagocytic-lymphohistiocystosis

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