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Journal articles on the topic 'Hemophagocytic Lymphohistiocytosis'

Author: Grafiati

Published: 4 June 2021

Last updated: 9 March 2023

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1

Nanwani, Poonam, Zoya Khan, and Ashok Yadav. "Secondary Hemophagocytic Lymphohistiocytosis." Annals of Pathology and Laboratory Medicine 6, no. 12 (December 24, 2019): A658–662. http://dx.doi.org/10.21276/apalm.2636.

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2

Jain, Dr Maganlal, Dr Arundhati Diwan, and Dr Rahul Shewale. "Hemophagocytic Lymphohistiocytosis - Revisited." International Journal of Scientific Research 3, no. 2 (June 1, 2012): 325–26. http://dx.doi.org/10.15373/22778179/feb2014/103.

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3

Martínez-Romera, Isabel, Marta Villa, Pilar Areal, Esther Rodrigo, Blanca Herrero, and Blanca López-Ibor. "Hemophagocytic Lymphohistiocytosis." Journal of Pediatric Hematology/Oncology 40, no. 2 (March 2018): e108-e110. http://dx.doi.org/10.1097/mph.0000000000000932.

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4

Chabchoub, Imen, Hela Boudabbous, Ines Maaloul, Rim Ben Abdelaziz, Amel Ben Chehida, Lobna Ayadi, Thouraya Kamoun, et al. "Hemophagocytic Lymphohistiocytosis." Journal of Pediatric Hematology/Oncology 42, no. 4 (May 2020): 310–12. http://dx.doi.org/10.1097/mph.0000000000001552.

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5

Major-Monfried, Hannah, Suzanne Friedman, and Scott Moerdler. "Hemophagocytic Lymphohistiocytosis." Pediatrics In Review 43, no. 10 (October 1, 2022): 596–98. http://dx.doi.org/10.1542/pir.2021-004985.

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6

Risma, Kimberly, and Michael B. Jordan. "Hemophagocytic lymphohistiocytosis." Current Opinion in Pediatrics 24, no. 1 (February 2012): 9–15. http://dx.doi.org/10.1097/mop.0b013e32834ec9c1.

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7

Hao, Jin-li, Ya-jing Zhang, Huai-xiu Yan, Fang Zhou, and Xin Chen. "Hemophagocytic lymphohistiocytosis." Discussion of Clinical Cases 1, no. 1 (October 15, 2014): 26. http://dx.doi.org/10.14725/dcc.v1n1p26.

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8

Skinner, Janice, Benedicta Yankey, and Brenda K. Shelton. "Hemophagocytic Lymphohistiocytosis." AACN Advanced Critical Care 30, no. 2 (June 15, 2019): 151–64. http://dx.doi.org/10.4037/aacnacc2019463.

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Hemophagocytic lymphohistiocytosis is a life-threatening condition associated with hyperinflammation and multiple organ dysfunction. It has many causes, symptoms, and outcomes. Early recognition is critical for treatment. Fever, cytopenias, coagulopathy, and hepatosplenomegaly are hallmark findings. Identifying the trigger event is crucial but challenging because of the varied presentations and infrequent provider experience. Diagnostic features include anemia, thrombocytopenia, neutropenia, elevated ferritin, hypertriglyceridemia, hypofibrinogenemia, hemophagocytosis (in bone marrow, spleen, or lymph nodes), low or absent natural killer cells, and elevated soluble interleukin 2 receptor assay. Primary treatment goals are eliminating the underlying trigger and suppressing hyperinflammation with steroids, immunoglobulins, or immunomodulators. Specific treatment includes corticosteroids, etoposide, and antithymocyte globulin followed by hematopoietic stem cell transplantation in patients with refractory or relapsing disease. Prompt immunochemical therapy is essential but often complicated by a high risk of treatment-related morbidity and disease recurrence. Despite these challenges, improvements in diagnostic technology and treatment have enhanced survival.

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9

Gowarty, Jasmine, Julie Oda, and Christian Cable. "Hemophagocytic lymphohistiocytosis." Baylor University Medical Center Proceedings 31, no. 3 (April 25, 2018): 350–51. http://dx.doi.org/10.1080/08998280.2018.1446877.

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10

Li, Jing, Qian Wang, Wenjie Zheng, Jie Ma, Wei Zhang, Wenze Wang, and Xinping Tian. "Hemophagocytic Lymphohistiocytosis." Medicine 93, no. 2 (March 2014): 100–105. http://dx.doi.org/10.1097/md.0000000000000022.

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11

Lim, Mary E., and Kwang Sik Kim. "HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS." Pediatric Infectious Disease Journal 18, no. 2 (February 1999): 154–55. http://dx.doi.org/10.1097/00006454-199902000-00014.

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12

Lachmann, Gunnar, Claudia Spies, Thomas Schenk, Frank M. Brunkhorst, Felix Balzer, and Paul La Rosée. "Hemophagocytic Lymphohistiocytosis." SHOCK 50, no. 2 (August 2018): 149–55. http://dx.doi.org/10.1097/shk.0000000000001048.

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13

Al-Samkari, Hanny, and Nancy Berliner. "Hemophagocytic Lymphohistiocytosis." Annual Review of Pathology: Mechanisms of Disease 13, no. 1 (January 24, 2018): 27–49. http://dx.doi.org/10.1146/annurev-pathol-020117-043625.

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14

Janka, G. E. "Hemophagocytic lymphohistiocytosis." Hematology 10, sup1 (September 2005): 104–7. http://dx.doi.org/10.1080/10245330512331390087.

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15

Mitra, Bhaskar, Ankit Shrivastav, Krishnendu Mukherjee, and Asit Ghosh. "Hemophagocytic lymphohistiocytosis." Hematology/Oncology and Stem Cell Therapy 2, no. 2 (April 2009): 367–70. http://dx.doi.org/10.1016/s1658-3876(09)50029-5.

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16

Filipovich, Alexandra H. "Hemophagocytic lymphohistiocytosis." Immunology and Allergy Clinics of North America 22, no. 2 (May 2002): 281–300. http://dx.doi.org/10.1016/s0889-8561(01)00009-1.

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17

Bafna, Vibha. "Hemophagocytic lymphohistiocytosis." Apollo Medicine 8, no. 4 (December 2011): 302–4. http://dx.doi.org/10.1016/s0976-0016(11)60012-4.

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18

Allen, Carl E., and Kenneth L. McClain. "Hemophagocytic lymphohistiocytosis." Paediatrics and Child Health 18, no. 3 (March 2008): 136–40. http://dx.doi.org/10.1016/j.paed.2007.12.009.

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19

Park, Sun Young, and Jae Min Lee. "Hemophagocytic Lymphohistiocytosis." Clinical Pediatric Hematology-Oncology 24, no. 1 (April 30, 2017): 11–20. http://dx.doi.org/10.15264/cpho.2017.24.1.11.

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20

Rosado, Flavia G. N., and Annette S. Kim. "Hemophagocytic Lymphohistiocytosis." American Journal of Clinical Pathology 139, no. 6 (June 2013): 713–27. http://dx.doi.org/10.1309/ajcp4zdkj4icouat.

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21

Raschke, Robert A., and Roxanne Garcia-Orr. "Hemophagocytic Lymphohistiocytosis." Chest 140, no. 4 (October 2011): 933–38. http://dx.doi.org/10.1378/chest.11-0619.

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22

Chellapandian, Deepak. "Hemophagocytic Lymphohistiocytosis." Immunology and Allergy Clinics of North America 40, no. 3 (August 2020): 485–97. http://dx.doi.org/10.1016/j.iac.2020.04.003.

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23

Biswal, N., Simi Shareef, P. Nalini, S. Srinivasan, and Debadutta Basu. "Hemophagocytic lymphohistiocytosis." Indian Journal of Pediatrics 66, no. 4 (July 1999): 632–35. http://dx.doi.org/10.1007/bf02727184.

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24

Narendra, A. M. V. R., G. Varun Kumar, A. Krishna Prasad, M. Shetty, Megha S. Uppin, and V. R. Srinivasan. "Hemophagocytic Lymphohistiocytosis." Indian Journal of Hematology and Blood Transfusion 30, no. 3 (November 22, 2012): 204–7. http://dx.doi.org/10.1007/s12288-012-0210-7.

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25

Trantham, Tanner, Jessica Auten, Benyam Muluneh, and Hendrik Van Deventer. "Ruxolitinib for the treatment of lymphoma-associated hemophagocytic lymphohistiocytosis: A cautionary tale." Journal of Oncology Pharmacy Practice 26, no. 4 (October 1, 2019): 1005–8. http://dx.doi.org/10.1177/1078155219878774.

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Introduction Hemophagocytic lymphohistiocytosis is a hyperinflammatory syndrome characterized by fever, hyperferritinemia, hypertriglyceridemia, hypofibrinogenemia, and pancytopenia. Three publications reported success with ruxolitinib, a Janus-associated kinase (JAK1/2) inhibitor. This therapy interrupts the production of cytokines associated with hemophagocytic lymphohistiocytosis, namely interferon-γ and interleukins 2, 6, and 10. Case report We administered ruxolitinib to two patients with lymphoma-associated hemophagocytic lymphohistiocytosis who had failed standard treatment with dexamethasone and etoposide. Management and outcome Patient #1 was started on ruxolitinib 10 mg BID, and titrated to 15 mg BID. All but two of the hemophagocytic lymphohistiocytosis criteria resolved within two weeks, and she was able to restart therapy for lymphoma. During her ruxolitinib taper, she again presented with relapsed hemophagocytic lymphohistiocytosis. She was taking 2.5 mg a day at the time. Despite salvage treatment, she died from the disease. Patient #2 was a diffuse large B-cell lymphoma patient who presented with hemophagocytic lymphohistiocytosis and was treated with chemoimmunotherapy and achieved a complete response (CR). Hemophagocytic lymphohistiocytosis symptoms relapsed, and he was treated with ruxolitinib. He developed relapsed lymphoma and unfortunately died. Discussion While treating the underlying lymphoma is a clear priority, the cytopenias and other symptoms of hemophagocytic lymphohistiocytosis complicate the delivery of this therapy. Hence, the use of ruxolitinib as a bridge to definitive therapy was appealing. However, we are concerned about the progression of lymphoma while these patients were taking ruxolitinib. Ruxolitinib may be controlling cytokine storm associated with hemophagocytic lymphohistiocytosis, while other aspects of the condition are progressing. Therefore, we would advise caution in its use in lymphoma-associated-hemophagocytic lymphohistiocytosis until more data are available.

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26

Hindi, Zakaria, Abdallah A. Khaled, and Ashraf Abushahin. "Hemophagocytic syndrome masquerading as septic shock: An approach to such dilemma." SAGE Open Medical Case Reports 5 (January 1, 2017): 2050313X1774630. http://dx.doi.org/10.1177/2050313x17746309.

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Introduction: Hemophagocytic syndrome or hemophagocytic lymphohistiocytosis is a rare condition characterized by excessive inflammation that is thought to be caused by the absence of normal downregulation of activated macrophages and lymphocytes. The treatment of hemophagocytic lymphohistiocytosis can depend on whether it is primary or secondary. In secondary hemophagocytic lymphohistiocytosis, the treatment can be directed according to the cause. In general, protocol HLH-94 (which consists of dexamethasone and etoposide in induction and maintenance) has been widely used as it has good outcomes. Hemophagocytic lymphohistiocytosis and septic shock largely overlap which can lead to refractory septic shock and death if not treated. Unfortunately, there is no clear approach for such dilemma. Thereby, we would like to present our case as it has a valuable approach to hemophagocytic lymphohistiocytosis in the setting of sepsis. Case description: A 60-year-old female, with history of hypertension, came with fever, productive cough, and dyspnea; she was admitted for acute exacerbation of chronic obstructive pulmonary disease and was transferred to intensive care unit for septic shock. The patient progressed to refractory septic shock with no focus of infection. After further investigations, detailed history raised the suspicion of hemophagocytic lymphohistiocytosis; a bone marrow biopsy was collected and confirmed the diagnosis. The patient was on methylprednisolone while waiting for other investigation results and improved markedly. After ruling out secondary causes of hemophagocytic lymphohistiocytosis, she was switched to protocol-94 and continued to improve. Conclusion: It should be emphasized that septic shock, with or without focus of infection, overlaps with hemophagocytic lymphohistiocytosis and can consequently lead to refractory septic shock and death. Thus, our aim of this case is to encourage further investigations, specifically for hemophagocytic lymphohistiocytosis in the setting of septic shock of unknown origin, to decrease mortality rate. More importantly, early initiation of immunosuppression therapy may be a crucial step before switching to hemophagocytic lymphohistiocytosis–specific treatment.

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27

Ouederni, Monia, MONIA BEN KHALED, Samia Rekaya, Ilhem Ben Fraj, Fethi Mellouli, and Mohamed Bejaoui. "A 9month-old-boy with atypical hemophagocytic lymphohistiocytosis." Mediterranean Journal of Hematology and Infectious Diseases 9, no. 1 (October 16, 2017): e2017057. http://dx.doi.org/10.4084/mjhid.2017.057.

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Hemophagocytic lymphohistiocytosis is a life-threatening hyperinflammation caused by uncontrolled proliferation of activated lymphocytes and histiocytes. Often, Hemophagocytic lymphohistiocytosis is an acquired syndrome. We report a case of a 9 month-old-boy presented with hepatosplenomegaly, severe anemia requiring repeated transfusions, thrombocytopenia, hypertriglyceridemia and very high hyperferritinemia. These clinical features of Hemophagocytic lymphohistiocytosis prompted a wide infectious and auto-immune request to be performed. After a wide diagnostic workup, he was referred to the immune hematologic unit, for hemophagocytic lymphohistiocytosis suspicion with unknown cause.

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28

Ouederni, Monia, MONIA BEN KHALED, Samia Rekaya, Ilhem Ben Fraj, Fethi Mellouli, and Mohamed Bejaoui. "A 9month-old-boy with atypical hemophagocytic lymphohistiocytosis." Mediterranean Journal of Hematology and Infectious Diseases 9, no. 1 (October 16, 2017): e2017057. http://dx.doi.org/10.4084/mjhid.2017.57.

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Hemophagocytic lymphohistiocytosis is a life-threatening hyperinflammation caused by uncontrolled proliferation of activated lymphocytes and histiocytes. Often, Hemophagocytic lymphohistiocytosis is an acquired syndrome. We report a case of a 9 month-old-boy presented with hepatosplenomegaly, severe anemia requiring repeated transfusions, thrombocytopenia, hypertriglyceridemia and very high hyperferritinemia. These clinical features of Hemophagocytic lymphohistiocytosis prompted a wide infectious and auto-immune request to be performed. After a wide diagnostic workup, he was referred to the immune hematologic unit, for hemophagocytic lymphohistiocytosis suspicion with unknown cause.

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29

McLean, Josef, Roia Katebian, Eugene Suh, Kamran Mirza, and Sachin Amin. "Neonatal Hemophagocytic Lymphohistiocytosis." NeoReviews 20, no. 6 (May 31, 2019): e316-e325. http://dx.doi.org/10.1542/neo.20-6-e316.

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30

Frikke, M. "Hemophagocytic Lymphohistiocytosis (HLH)." PEDIATRICS 114, no. 4 (October 1, 2004): 1131–32. http://dx.doi.org/10.1542/peds.2004-1491.

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31

Haytoglu, Zeliha, Nalan Yazici, and Ayse Erbay. "Secondary Hemophagocytic Lymphohistiocytosis." Journal of Pediatric Hematology/Oncology 39, no. 2 (March 2017): e106-e109. http://dx.doi.org/10.1097/mph.0000000000000740.

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32

Herman, T. E., and M. J. Siegel. "Familial hemophagocytic lymphohistiocytosis." Journal of Perinatology 30, no. 5 (April 29, 2010): 363–65. http://dx.doi.org/10.1038/jp.2010.25.

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33

Rice, Lawrence, and Jose Pacheco. "Adult Hemophagocytic Lymphohistiocytosis." Critical Care Medicine 44, no. 11 (November 2016): 2119–21. http://dx.doi.org/10.1097/ccm.0000000000001946.

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34

Henter, Jan-Inge, Maurizio Aricò, Göran Elinder, Shinsaku Imashuku, and Gritta Janka. "FAMILIAL HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS." Hematology/Oncology Clinics of North America 12, no. 2 (April 1998): 417–33. http://dx.doi.org/10.1016/s0889-8588(05)70520-7.

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35

Canna, Scott W., and Rebecca A. Marsh. "Pediatric hemophagocytic lymphohistiocytosis." Blood 135, no. 16 (April 16, 2020): 1332–43. http://dx.doi.org/10.1182/blood.2019000936.

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Abstract Hemophagocytic lymphohistiocytosis (HLH) is a syndrome describing patients with severe systemic hyperinflammation. Characteristic features include unremitting fever, cytopenias, hepatosplenomegaly, and elevation of typical HLH biomarkers. Patients can develop hepatitis, coagulopathy, liver failure, central nervous system involvement, multiorgan failure, and other manifestations. The syndrome has a high mortality rate. More and more, it is recognized that while HLH can be appropriately used as a broad summary diagnosis, many pediatric patients actually suffer from an expanding spectrum of genetic diseases that can be complicated by the syndrome of HLH. Classic genetic diseases in which HLH is a typical and common manifestation include pathogenic changes in familial HLH genes (PRF1, UNC13D, STXBP2, and STX11), several granule/pigment abnormality genes (RAB27A, LYST, and AP3B1), X-linked lymphoproliferative disease genes (SH2D1A and XIAP), and others such as NLRC4, CDC42, and the Epstein-Barr virus susceptibility diseases. There are many other genetic diseases in which HLH is an infrequent complication of the disorder as opposed to a prominent manifestation of the disease caused directly by the genetic defect, including other primary immune deficiencies and inborn errors of metabolism. HLH can also occur in patients with underlying rheumatologic or autoinflammatory disorders and is usually designated macrophage activation syndrome in those settings. Additionally, HLH can develop in patients during infections or malignancies without a known (or as-yet-identified) genetic predisposition. This article will attempt to summarize current concepts in the pediatric HLH field as well as offer a practical diagnostic and treatment overview.

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36

Millsop, Jillian W., Baran Ho, Maija Kiuru, Maxwell A. Fung, and Victoria R. Sharon. "Cutaneous Hemophagocytic Lymphohistiocytosis." JAMA Dermatology 152, no. 8 (August 1, 2016): 950. http://dx.doi.org/10.1001/jamadermatol.2016.0978.

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37

Raka, S., P. Nayar, R. Godbole, and R. Manchanda. "Familial hemophagocytic lymphohistiocytosis." Indian Journal of Hematology and Blood Transfusion 25, no. 2 (June 2009): 78–80. http://dx.doi.org/10.1007/s12288-009-0018-2.

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38

Lachman, Mary F., Edward H. Vogel, and Kitai Kim. "Familial hemophagocytic lymphohistiocytosis." Diagnostic Cytopathology 4, no. 2 (March 1988): 121–24. http://dx.doi.org/10.1002/dc.2840040208.

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39

Degar, Barbara. "Familial Hemophagocytic Lymphohistiocytosis." Hematology/Oncology Clinics of North America 29, no. 5 (October 2015): 903–13. http://dx.doi.org/10.1016/j.hoc.2015.06.008.

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40

Fernández-Pérez, Evans R., and Abdi A. Jama. "Nonfamilial hemophagocytic lymphohistiocytosis." American Journal of Hematology 82, no. 12 (2007): 1118–19. http://dx.doi.org/10.1002/ajh.21025.

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41

Filipovich, Alexandra H. "Hemophagocytic Lymphohistiocytosis and Other Hemophagocytic Disorders." Immunology and Allergy Clinics of North America 28, no. 2 (May 2008): 293–313. http://dx.doi.org/10.1016/j.iac.2008.01.010.

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42

Scalzone, Maria, Antonio Ruggiero, Stefano Mastrangelo, Giovanna Trombatore, Vita Ridola, Palma Maurizi, and Riccardo Riccardi. "Hemophagocytic lymphohistiocytosis and visceral leishmaniasis in children: case report and systematic review of literature." Journal of Infection in Developing Countries 10, no. 01 (January 31, 2016): 103–8. http://dx.doi.org/10.3855/jidc.6385.

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Hemophagocytic lymphohistiocytosis is a potentially fatal disorder resulting from excessive activation and non-malignant proliferation of T lymphocytes and macrophages. Neoplasms, autoimmune disorders and systemic infections can cause secondary hemophagocytic syndrome. The association of hemophagocytic syndrome and visceral leishmaniasis is rarely found in childhood. We report a case of an infant affected by hemophagocytic lymphohistiocytosis secondary to visceral leishamniasis and describe all cases of hemophagocytic syndrome associated with visceral leishamniasis in childhood reported in literature, focusing on clinical manifestation, diagnosis and treatment.

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43

Costa, Andreia Sofia, Anusca Paixão, Henrique Santos, and Fernando Salvador. "Linfohistiocitose Hemofagocítica Secundária a Infeção por Epstein-Barr: Raridade e Gravidade num Adulto Imunocompetente." Acta Médica Portuguesa 32, no. 1 (February 1, 2019): 78. http://dx.doi.org/10.20344/amp.9474.

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Hemophagocytic lymphohistiocytosis is a rare, aggressive and life-threatening syndrome, characterized by an excessive immune activation. It is triggered by multiple stimuli, with infections having an important role. The most common infectious trigger is viral infection, particularly by Epstein-Barr virus. Coagulation disorders are common in hemophagocytic lymphohistiocytosis and disseminated intravascular coagulation can be present in severe cases. We report a clinical case of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis, complicated with disseminated intravascular coagulation which evolved favorably with only supportive therapy and without specific treatment.

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El-Sayed, Zeinab A., Rasha H. El-Owaidy, Mohammed A. Khamis, and Ahmed R. Rezk. "Screening of hemophagocytic lymphohistiocytosis in children with severe sepsis in pediatric intensive care." Science Progress 104, no. 3 (July 2021): 003685042110440. http://dx.doi.org/10.1177/00368504211044042.

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Background: We sought to screen for clinical and laboratory features of hemophagocytic lymphohistiocytosis among pediatric patients with severe sepsis. Methods: We conducted a retrospective study that analyzed the clinical and laboratory data of 70 pediatric patients who died of severe sepsis. Medical records were revised for the presence of fever, splenomegaly, pancytopenia, hyperferritinemia, hypertriglyceridemia, and hypofibrinogenemia. Soluble CD25 was measured in stored samples. Results: Patients’ ages ranged between 0.5 and 11 years with median (interquartile range) 2 (1–5). All patients had fever (≥38.5 °C) and pancytopenia, 58 (82.9%) hepatosplenomegaly, 36 (51.4%) lymphadenopathy, 37 (52.9%) had ferritin >500 ng/ml, 20 (28.6%) had fibrinogen <1.5 mg/ml, 14 (20%) had fasting triglycerides >264 mg/dl while 5 (7.1%) had soluble CD25 >2400 U/ml. Twenty-five (35.7%) patients fulfilled at least 5/6 of the hemophagocytic lymphohistiocytosis-2004 diagnostic criteria. Multivariate backward binary logistic regression analysis revealed lymphadenopathy as an independent predictor for hemophagocytic lymphohistiocytosis criteria fulfilment with odds ratio of 23.9. Fibrinogen had the best performance in discriminating hemophagocytic lymphohistiocytosis fulfilling from non-fulfilling groups (cut-off value: <1.8 mg/ml), followed by ferritin/erythrocyte sedimentation rate ratio (cut-off value: >17). Conclusion: There is a significant clinical and laboratory overlap between hemophagocytic lymphohistiocytosis and severe sepsis, making the syndromes difficult to distinguish. The use of current hemophagocytic lymphohistiocytosis-2004 diagnostic criteria should be applied cautiously in those patients.

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45

Parizhskaya, Maria, Jorge Reyes, and Ronald Jaffe. "Hemophagocytic Syndrome Presenting as Acute Hepatic Failure in Two Infants: Clinical Overlap with Neonatal Hemochromatosis." Pediatric and Developmental Pathology 2, no. 4 (July 1999): 360–66. http://dx.doi.org/10.1007/s100249900135.

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Two patients with hemophagocytic lymphohistiocytosis who presented with acute liver failure are reported. Both presented with fever, hepatosplenomegaly, markedly elevated liver function tests, abnormal coagulation profiles, and an increase in serum ferritin. Both infants were diagnosed with neonatal hemochromatosis based on a clinical picture of hepatic insufficiency with hyperferritinemia and were referred for liver transplantation. The first patient died of liver failure and septicemia before transplantation. Review of autopsy material revealed a hepatitis-like pattern and extensive infiltration of liver and other organs including bone marrow by histiocytes, some of which were hemophagocytic. The second patient underwent liver transplantation but died 44 days thereafter from progressive hemophagocytic lymphohistiocytosis. Examination of the resected liver demonstrated a hepatitis-like pattern, proliferation of histiocytes, and hemophagocytosis, and the bone marrow revealed hemophagocytic histiocytosis. Hemophagocytosis recurred in the allograft. Hepatic manifestations are common in hemophagocytic lymphohistiocytosis and overt hepatic failure may occur, but initial presentation as fulminant hepatic failure is not well recognized. Elevated serum ferritin can make the distinction from neonatal hemochromatosis and other forms of neonatal liver failure difficult. Hemophagocytic lymphohistiocytosis should be considered in the differential diagnosis of neonatal liver disease, especially when it is accompanied by cytopenias.

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46

Smith, Simon, Azhar Mohamed Munas, and Josh Hanson. "Hemophagocytic Lymphohistiocytosis Complicating Melioidosis." American Journal of Tropical Medicine and Hygiene 99, no. 3 (September 5, 2018): 557–58. http://dx.doi.org/10.4269/ajtmh.18-0331.

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47

de Jesus, Mikhail, Amanda Lopez, Jevin Yabut, Stephanie Vu, Madhuri Manne, Lauren Ibrahim, and Rahul Mutneja. "Anaplasmosis-induced hemophagocytic lymphohistiocytosis." Baylor University Medical Center Proceedings 35, no. 3 (March 17, 2022): 379–81. http://dx.doi.org/10.1080/08998280.2022.2039046.

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48

Bagdasaryan, N. G., G. S. Ayvazyan, L. A. Avetisyan, N. R. Mnatsakanyan, and L. T. Aleksanyan. "Hemophagocytic Lymphohistiocytosis in Children." Doctor.Ru 20, no. 3 (2021): 66–69. http://dx.doi.org/10.31550/1727-2378-2021-20-3-66-69.

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Objective of the Paper: To present a clinical case of a 2.5-year girl hospitalised with severe pneumonia with associated hemophagocytic lymphohistiocytosis (HLH). Key Points. HLH is a syndrome, the clinical representation of which is a system sepsis-like inflammatory reaction; in the majority of cases, this condition is hardly diagnosed by paediatricians. Associated pancytopenia and hyperferritinaemia are of diagnostic importance. Here we present a clinical case of a 2.5-year girl who was hospitalised with severe pneumonia and associated HLH. As there is no common approach to the management of HLH, we summoned a board of doctors and decided to start steroid therapy (dexamethasone) and IV immunoglobulin (Octagam), and if there was no effect from therapy within a week, cytostatic agents would be added. The therapy was initiated promptly after macrophages had been found in bone marrow smear. Clinical and paraclinic improvements were noted on day 5, so aggressive immunosuppresion was not initiated. In a month there were no signs of organ dysfunction; the values normalised, and the child was discharged from the inpatient clinic. Conclusion. HLH is associated with high mortality, therefore, early diagnosis and therapy are critical. The specificity of this case is that we managed to promptly diagnose the condition, initiated treatment and achieved complete remission without aggressive immunosuppresion. Keywords: hemophagocytic lymphohistiocytosis, macrophages, pancytopenia.

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Høyvoll, Liv R., Yngvar Fløisand, Hilde Lang Orrem, Ragnar Gunnarsson, Linn Landrø, Trine Brevig, Peter Gaustad, and Ingvild Nordøy. "Hemophagocytic lymphohistiocytosis in leprosy." Leprosy Review 86, no. 4 (December 1, 2015): 403–6. http://dx.doi.org/10.47276/lr.86.4.403.

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Kam, Kai-qian, Shui Yen Soh, and Rajat Bhattacharyya. "Dengue-associated Hemophagocytic Lymphohistiocytosis." Journal of Pediatric Hematology/Oncology 40, no. 6 (August 2018): e377-e379. http://dx.doi.org/10.1097/mph.0000000000001000.

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