Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder with incidence 1 in 50.000 to 150.000 live births. HLH has high fatality rate and poor prognosis, therefore early recognition and diagnosis are necessary. Hereby we aimed to describe the clinical, examination and management aspects of HLH. An eight-month-old girl came with bloating stomach for 1 week and fever since 2 weeks before admission. She also had chronic suppurated otitis media in previous month. Patient has twin sibling, whom in healthy condition. Patient looked pale with distended abdomen. We found enlarge liver and spleen which were palpabled 4 cm under the arch of costae and Schuffner IV, respectively. Multiple purpura were found on trunk, head and extremities. Laboratory tests revealed severe normochromic normocytic anemia, neutropenia, severe thrombocytopenia, hyperferritinemia and reactive anti CMV IgG. Blood culture resulted no growth. Urine culture revealed the growth of Klebsiella pneumonia, first ear swab culture showed an isolated Pseudomonas aeruginosa and second ear swab culture with Klebsiella pneumonia. The histopathologic examination from bone marrow aspiration revealed hemophagocytic histiocyte. Patient was given broad spectrum antibiotics and supportive therapy and oral dexamethasone after HLH was diagnosed. The patient was pronounced dead during second admission. HLH can be considered as one of the differential diagnosis in children with prolong fever, hepatosplenomegaly and cytopenia. Appropriate treatment protocol should be taken to avoid any complications.
| Published in | World Journal of Medical Case Reports (Volume 2, Issue 2) |
| DOI | 10.11648/j.wjmcr.20210202.11 |
| Page(s) | 19-24 |
| Creative Commons |
This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited. |
| Copyright |
Copyright © The Author(s), 2021. Published by Science Publishing Group |
Children, Secondary HLH, Non-familial HLH
| [1] | McLean, J., Katebian, R., Suh, E., Mirza, K., Amin, S. (2019). Neonatal Hemophagocytic Lymphohistiocytosis. Neo Reviews 20 (6): 317-325. |
| [2] | Morimoto, A., Nazakawa, Y., Ishii, E. (2016). Hemophagocytic lymphohistiocytosis: Pathogenesis, diagnosis, and management. Pediatric International. 58: 817-825. |
| [3] | Wang, A., Pope, S., Weinstein, J., Yu, S., Zhang, C., Booth C., Medxhitov, R. (2016). Specific sequences of infectious challenge lead to secondary hemophagocytic lymphohistiocytosis-like disease in mice. PNAS: 1-16. |
| [4] | Allen, C., McClain, K. (2015). Pathophysiology and epidemiology of hemophagocytic lymphohistiocytosis. Hematology: 177-182. |
| [5] | Ishii, E., Ohga, S., Imashuku, S., Yasukawa, M., Tsuda, M., Miura, I., Yamamoto, K., Hirouichi, H. (2017). Nationwide Survey of Hemophagocytic Lymphohistiocytosis in Japan. Int J Hematology. 86: 58-65. |
| [6] | Dewi, Y., Sukorini, U., Budi, M., Intansari, U. (2015). Hemophagocytic Lymphohistuocytosis (HLH) pada Severe Systemic Lupus Erythematosus (SLE) dengan Autoimmune Hemolytic Anemia (AIHA) refracter. OJS Universitas Gadjah Mada. |
| [7] | Canna, S., Marsh, R. (2010). Rare Systemic Hematologic Disorders: Pediatric hemophagocytic lymphohistiocytosis. The American Society of Hematology. 15 (16): 1322-1343. |
| [8] | Garcia, C., Haye, K., Gahig, T. (2016). Hemophagocytic Lymphohistiocytosis (HLH): A Case Series and Review. American Journal of Medical Case Reports. 4 (3): 74-79. |
| [9] | Cascio, A., Pernice, K., Barberi, G., Biondo, C., Beniati, C., Mancuso, G., Morales, R., Iaria, C. (2012). Secondary hemophagocytic lymphohistiocytosis in zoonoses. A systematic review. European Reiview for Medical and Pharmacological Sciences. 16: 1324-1337. |
| [10] | Castillo. L., Carcillo. J. (2009). Secondary hemophagocytic lymphohistiocytosis and severe sepsis/systemic inflammatory response syndrome/multiorgan dysfunction syndrome/macrophage activation syndrome share common intermediate phenotypes on a spectrum of inflammation. Pediatr Crit Care Med. 10 (3): 367-372. |
| [11] | Brisse, E., Wouters, C., Andrei, G., Matthys, P. (2017). How viruses contribute to the Pathogenesis of Hemophagocytic Lymphohistiocytosis. Frontiers in immunology. 8 (1): 102-110. |
| [12] | Tumian, B., Wong, C. (2015). Pregnancy-related hemophagocytic lymphohistiocytosis associated with cytomegalovirus infection: A diagnostic and therapeutic challenge. Taiwanese Journal of Obstetrics & Gynecology. 54: 432-437. |
| [13] | Zerah, M., Witt, C. (2015). Cutaneous Findings in Hemophagocytic Lymphohistiocytosis. Dermatology. 230: 234-243. |
| [14] | Carthy, L., Fernandez, K., Antony, R. (2017). Challenges in the Diagnosis and Management of Pediatric Hemophagocytic Lymphohistiocytosis. Clinical Pediatrics. 1-7. |
| [15] | Grange, S., Buchonnet, G., Macari, E., Beduncau, G., Carpentier, D., Dehay, J., Girault, C. (2016). The Use of Ferritin to Identify Critically Ill Patients With Secondary Hemophagocytic Lymphohistiocytosis. Critical Care Medicine. 44 (11): 1045-1051. |
| [16] | Henter, J., Home, A., Arico, M., Egeler, M., Imashuku, S., Ledich, S., Clain, K. (2006). REVIEW HLH-2004: Diagnostic and Therapeutic Guidelines for Hemophagocytic Lymphohistiocytosis. Pediatr Blood Center. |
| [17] | Yanagisawa, R., Nakazawa, Y., Matsuda, K., Yasumi, T., Kanegane, H., Marimoto, A., Imaizumi, M. (2018). Outcomes in children with hemophagocytic lymphohistiocytosis treated using HLH-2004 protocol in Japan. Int J Hemato. 125-131. |
| [18] | Ehi, S., Astigaraga, I., Greenwood, T., Hiner, M., Horne, A., Henka, G., Rosee, P. (2018). Recommendations for the Use of Etoposide-Based Therapy and Bone Marrow Transplantation for the Treatment of HLH: Consensus Statements by the HLH Steering Committee of the Histiocyte Society. Am Ascc Allergy. 321-333. |
| [19] | Pan, H., Huo, Y., Sun, L. (2019). Comparison between clinical features and prognosis of malignancy- and nonmalignancy–associated pediatric hemophagocytic lymphohistiocytosis. BMC Pediatrics. 19: 468-475. |
APA Style
Hanzelina, Ketut Ariawati, Anak Agung Ngurah Ketut Putra Widnyana. (2021). Hemophagocytic Lymphohistiocytosis in an 8-Month-Old Baby. World Journal of Medical Case Reports, 2(2), 19-24. https://doi.org/10.11648/j.wjmcr.20210202.11
ACS Style
Hanzelina; Ketut Ariawati; Anak Agung Ngurah Ketut Putra Widnyana. Hemophagocytic Lymphohistiocytosis in an 8-Month-Old Baby. World J. Med. Case Rep. 2021, 2(2), 19-24. doi: 10.11648/j.wjmcr.20210202.11
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Download@article{10.11648/j.wjmcr.20210202.11,
author = {Hanzelina and Ketut Ariawati and Anak Agung Ngurah Ketut Putra Widnyana},
title = {Hemophagocytic Lymphohistiocytosis in an 8-Month-Old Baby},
journal = {World Journal of Medical Case Reports},
volume = {2},
number = {2},
pages = {19-24},
doi = {10.11648/j.wjmcr.20210202.11},
url = {https://doi.org/10.11648/j.wjmcr.20210202.11},
eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.wjmcr.20210202.11},
abstract = {Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder with incidence 1 in 50.000 to 150.000 live births. HLH has high fatality rate and poor prognosis, therefore early recognition and diagnosis are necessary. Hereby we aimed to describe the clinical, examination and management aspects of HLH. An eight-month-old girl came with bloating stomach for 1 week and fever since 2 weeks before admission. She also had chronic suppurated otitis media in previous month. Patient has twin sibling, whom in healthy condition. Patient looked pale with distended abdomen. We found enlarge liver and spleen which were palpabled 4 cm under the arch of costae and Schuffner IV, respectively. Multiple purpura were found on trunk, head and extremities. Laboratory tests revealed severe normochromic normocytic anemia, neutropenia, severe thrombocytopenia, hyperferritinemia and reactive anti CMV IgG. Blood culture resulted no growth. Urine culture revealed the growth of Klebsiella pneumonia, first ear swab culture showed an isolated Pseudomonas aeruginosa and second ear swab culture with Klebsiella pneumonia. The histopathologic examination from bone marrow aspiration revealed hemophagocytic histiocyte. Patient was given broad spectrum antibiotics and supportive therapy and oral dexamethasone after HLH was diagnosed. The patient was pronounced dead during second admission. HLH can be considered as one of the differential diagnosis in children with prolong fever, hepatosplenomegaly and cytopenia. Appropriate treatment protocol should be taken to avoid any complications.},
year = {2021}
}
TY - JOUR T1 - Hemophagocytic Lymphohistiocytosis in an 8-Month-Old Baby AU - Hanzelina AU - Ketut Ariawati AU - Anak Agung Ngurah Ketut Putra Widnyana Y1 - 2021/05/31 PY - 2021 N1 - https://doi.org/10.11648/j.wjmcr.20210202.11 DO - 10.11648/j.wjmcr.20210202.11 T2 - World Journal of Medical Case Reports JF - World Journal of Medical Case Reports JO - World Journal of Medical Case Reports SP - 19 EP - 24 PB - Science Publishing Group SN - 2994-726X UR - https://doi.org/10.11648/j.wjmcr.20210202.11 AB - Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder with incidence 1 in 50.000 to 150.000 live births. HLH has high fatality rate and poor prognosis, therefore early recognition and diagnosis are necessary. Hereby we aimed to describe the clinical, examination and management aspects of HLH. An eight-month-old girl came with bloating stomach for 1 week and fever since 2 weeks before admission. She also had chronic suppurated otitis media in previous month. Patient has twin sibling, whom in healthy condition. Patient looked pale with distended abdomen. We found enlarge liver and spleen which were palpabled 4 cm under the arch of costae and Schuffner IV, respectively. Multiple purpura were found on trunk, head and extremities. Laboratory tests revealed severe normochromic normocytic anemia, neutropenia, severe thrombocytopenia, hyperferritinemia and reactive anti CMV IgG. Blood culture resulted no growth. Urine culture revealed the growth of Klebsiella pneumonia, first ear swab culture showed an isolated Pseudomonas aeruginosa and second ear swab culture with Klebsiella pneumonia. The histopathologic examination from bone marrow aspiration revealed hemophagocytic histiocyte. Patient was given broad spectrum antibiotics and supportive therapy and oral dexamethasone after HLH was diagnosed. The patient was pronounced dead during second admission. HLH can be considered as one of the differential diagnosis in children with prolong fever, hepatosplenomegaly and cytopenia. Appropriate treatment protocol should be taken to avoid any complications. VL - 2 IS - 2 ER -
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