Rare Case of Post-Infectitous Hemophagocytic Lymphohistiocytosis resenting as Panniculitis & Multi-Organ Dysfunction Syndrome

Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening hyperinflammatory syndrome caused by excessive activation of the immune system. It often mimics severe infections or sepsis and poses a significant diagnostic challenge. We present a rare case of HLH in a 40-year-old female with an unusual presentation involving panniculitis, jaundice, and multi-organ dysfunction. Prompt recognition of persistent cytopenias and systemic inflammation led to the diagnosis through bone marrow biopsy. The patient showed significant improvement following corticosteroid therapy. This case highlights the importance of maintaining a high index of suspicion for HLH in patients with prolonged systemic inflammation and cytopenias. 1. Mellissa R George, Hemophagocytic Lymphohistiocytosis: Review of Etiologies and Management. Journal of Blood Medicine. J Blood Med. 2014 Jun 12:5:69- 86. Doi: 10.2147/JBM.S46255. eCollection 2014. 2. Flavia G N Rosado, MD, Annete S Kim, MD, PPHD. Hemophagocytic Lymphohistiocytosis: An update on Diagnosis and Pathogenesis. American Journal of Clinical Pathology, Volume 139, Issue 6, June 2013 Page 713-727. 3. UpToDate - www.uptodate.com/contents/clinicalfeatures-and-diagnosis-ofhemophagocytic-lymphohistiocytosis/ 4. Robert A Schwartz, MD. Medscape Update Feb 26, 2021.

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Journal
American Journal of Case Reports and Clinical Images
Published
2025-01-01
DOI
https://doi.org/10.51521/ajcrci.2025.22.118
Primary Topic
Autoimmune and Inflammatory Disorders Research
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article
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article

Rare Case of Post-Infectitous Hemophagocytic Lymphohistiocytosis resenting as Panniculitis & Multi-Organ Dysfunction Syndrome

Steffi Thomas Sebin, Dr Nilesh Wasekar
American Journal of Case Reports and Clinical Images
Autoimmune and Inflammatory Disorders Research
article

Rare Case of Post-Infectitous Hemophagocytic Lymphohistiocytosis resenting as Panniculitis & Multi-Organ Dysfunction Syndrome

Steffi Thomas Sebin, Dr Nilesh Wasekar
article en

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening hyperinflammatory syndrome caused by excessive activation of the immune system. It often mimics severe infections or sepsis and poses a significant diagnostic challenge. We present a rare case of HLH in a 40-year-old female with an unusual presentation involving panniculitis, jaundice, and multi-organ dysfunction. Prompt recognition of persistent cytopenias and systemic inflammation led to the diagnosis through bone marrow biopsy. The patient showed significant improvement following corticosteroid therapy. This case highlights the importance of maintaining a high index of suspicion for HLH in patients with prolonged systemic inflammation and cytopenias. 1. Mellissa R George, Hemophagocytic Lymphohistiocytosis: Review of Etiologies and Management. Journal of Blood Medicine. J Blood Med. 2014 Jun 12:5:69- 86. Doi: 10.2147/JBM.S46255. eCollection 2014. 2. Flavia G N Rosado, MD, Annete S Kim, MD, PPHD. Hemophagocytic Lymphohistiocytosis: An update on Diagnosis and Pathogenesis. American Journal of Clinical Pathology, Volume 139, Issue 6, June 2013 Page 713-727. 3. UpToDate - www.uptodate.com/contents/clinicalfeatures-and-diagnosis-ofhemophagocytic-lymphohistiocytosis/ 4. Robert A Schwartz, MD. Medscape Update Feb 26, 2021.

American Journal of Case Reports and Clinical ImagesVol. 02(02)
Dr. D. Y. Patil Medical College, Hospital and Research Centre (IN)
Openalex Percentile: Top 68%
Autoimmune and Inflammatory Disorders Research
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Rare Case of Post-Infectitous Hemophagocytic Lymphohistiocytosis resenting as Panniculitis & Multi-Organ Dysfunction Syndrome — Steffi Thomas Sebin, Dr Nilesh Wasekar · American Journal of Case Reports and Clinical Images (2025) | TGRS Research Map | TGRS