A Case Report of Hemophagocytic Lymph Histiocytosis in a Young Male

Putu Dwi Pradnya Ardhaneswari, Ni Kadek Mulyantari, I. Nyoman Wande, Anak Agung Wiradwi Lestari


Hemophagocytic Lymph Histiocytosis (HLH) is a clinical syndrome affected by excessive immune stimulation and tissue hyperinflammation, which is relatively underdiagnosed because of its various clinical manifestations. This case described a young male whose body temperature measured at 39°C and only dropped briefly due to a fever reducer. He had a painless inflammation in the neck during the fever. Cold and stuffy nose emerged before the fever appeared. Moreover, stuffy nose caused snoring sleep. The patient also faced bleeding gums and nosebleeds. However, he did not experience body ache or weight loss. Various clinical tests, such as clinical chemistry laboratory tests, blood and bone marrow tests, and radiological tests were conducted. Clinical chemistry test revealed a decrease in albumin levels. Additionally, peripheral blood test showed a smear of microcytic hypochromic anemia, leukopenia and thrombocytopenia, while bone marrow examination showed a process of hem phagocytosis/metabolic disease. Furthermore, radiological tests showed no abnormalities.


Keywords: hemophagocytic lymph histiocytosis, immune stimulation, tissue hyperinflammation, young male.



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SIDDAIAHGARI S, AGARWAL S, & MOODAHADU L S. Hemophagocytic lymph histiocytosis: A review. Journal of Blood Disorder and Transfusion, 2016, 7(4): 121–126. https://doi.org/10.4172/2155-9864.1000363

UNAL S. Hemophagocytic lymph histiocytosis: An update to diagnosis and management. Acta Medica Cordoba, 2014, 3(2): 29–32.

SALUNKE B, SAVARKAR S, & PATIL V P. Hemophagocytic syndrome-An approach to the management. Indian Journal of Critical Care Medicine, 2019, 23(6): 191-196. https://doi.org/10.5005/jp-journals-10071-23251

MACHACZKA M, VAKTNAS J, KLIMKOWSKA M, & HAGGLUND H. Malignancy-associated hemophagocytic lymph histiocytosis in adults: A retrospective population-based analysis from a single center. Leukemia & Lymphoma, 2011, 52(4): 613-619. https://doi.org/10.3109/10428194.2010.551153

ROSADO F G N, KIM A S, & ROUTT H G. Hemophagocytic lymph histiocytosis: An update on diagnosis and pathogenesis. American Journal of Clinical Pathology, 2018, 139(6): 19–27.

GRZYBOWSKI B, VISHWANATH V. A, & HORNE A. Hemophagocytic lymph histiocytosis: A diagnostic conundrum. Journal of Pediatric Neurosciences, 2017, 27: 114–122. https://doi.org/10.4103/jpn.JPN_140_16

ZHANG L, ZHOU J, & SOKOL L. Hereditary and acquired hemophagocytic lymph histiocytosis cancer control. Acta Medica Cordoba, 2018, 1(1): 9–20. https://doi.org/10.1177/107327481402100406

LI J, WANG Q, ZHENG W, MA J, ZHANG W, WANG W, & TIAN X. Hemophagocytic lymph histiocytosis: Clinical analysis of 103 adult patients. Medicine (Baltimore), 2014, 93(2): 100-105.

ABOULEISH Y, AHMAD Q, & MARIK P. The hunt for hemophagocytic lymph histiocytosis using procalcitonin. Chest Journal of Hematology, 2020, 1(1): 72–83. https://doi.org/10.1016/j.chest.2020.08.905

GEORGE M. R. Hemophagocytic lymph histiocytosis: Review of etiologies and management. Journal of Blood Medicine, 2014, 12(2): 91–108. https://doi.org/10.2147/JBM.S46255

HANZELINA, ARIAWATI K, & WIDNYANA A A N K P. Hemophagocytic lymph histiocytosis in an 8-month-old baby. World Journal of Medical Case, 2021, 5(1): 178–186.

SHARMA S, & DAWSON L. Pancytopenia induced by secondary hemophagocytic lymph histiocytosis: A rare, overlocked dreadful complication of plasmodium vivax. Tropical Parasitology, 2020, 10(1): 50-55. https://doi.org/10.4103/tp.TP_44_19

AKSIONAU A, & WEI E X. Accuracy of the criteria for hemophagocytic lymph histiocytosis. International Journal of Clinical and Experimental Pathology, 2020, 13(12): 3139-3148.

EMILIE J F, ABLA O, FRAITAG S, & ARITLE T G. Revised classification of histiocytosis and neoplasms of the macrophage-dendritic cell lineages. Blood, 2016, 127(22): 2672-2681. https://doi.org/10.1182/blood-2016-01-690636

HENTER J I, HORNE A, ARICO M, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymph histiocytosis. Pediatric Blood & Cancer, 2007, 48(2): 124-131.

JORDAN M B, ALLEN C E. WEITZMAN S, et al. How I treat hemophagocytic lymph histiocytosis. Blood, 2011, 118(3): 4041-4052.


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