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1

Costello, R., V. Baccini, K. Mazodier, G. Kaplanski, T. Le Treut, and G. Sébahoun. "Lymphohistiocytose hémophagocytaire." EMC - Hématologie 2, no. 3 (January 2007): 1–9. http://dx.doi.org/10.1016/s1155-1984(07)48472-2.

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2

Bouchair, N., D. Belamri, H. Sehab, W. Boutabia, A. Belghersa, J. Bounour, A. Bastandji, F. Benchikh, and M. Yaiche. "P418 - Lymphohistiocytose familiale. Une nouvelle observation." Archives de Pédiatrie 17, no. 6 (June 2010): 154–55. http://dx.doi.org/10.1016/s0929-693x(10)70812-5.

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3

Galambrun, C., JL Stephan, A. Fischer, N. Philippe, P. Bordigoni, G. Michel, F. Méchinaud, H. Rubie, and JP Vannier. "Lymphohistiocytose familiale : étude rétrospective de 41 observations." Archives de Pédiatrie 5, no. 6 (June 1998): 701. http://dx.doi.org/10.1016/s0929-693x(98)80190-5.

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4

Zoubeidi, H., F. Daoud, L. Baili, Z. Aydi, B. Ben Dhaou, M. Maroub, and F. Boussema. "Étude de la lymphohistiocytose hémophagocytaire de l’adulte." La Revue de Médecine Interne 35 (December 2014): A102—A103. http://dx.doi.org/10.1016/j.revmed.2014.10.166.

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5

Zriba, S., K. Kacem, F. Daoud, F. Lajili, S. B’Chir Hamzaoui, H. Ghédira, B. Ben Dhaou, et al. "La lymphohistiocytose hémophagocytaire de l’adulte : à propos de 39 cas." La Revue de Médecine Interne 35 (June 2014): A147. http://dx.doi.org/10.1016/j.revmed.2014.03.240.

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6

Dufourcq Lagelouse, R., F. Le Deist, A. Fisher, and G. de Saint Basile. "Altération du gène codant pour la perforine dans la lymphohistiocytose familiale." médecine/sciences 15, no. 12 (1999): 1479. http://dx.doi.org/10.4267/10608/1301.

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7

Dilies, A. C., L. Diaz, C. Lesouder, P. Guilpain, J. L. Faillie, P. Palassin, and A. Maria. "Lymphohistiocytose hémophagocytaire associée aux inhibiteurs du point de contrôle immunologique : étude de pharmacovigilance." La Revue de Médecine Interne 42 (June 2021): A42—A43. http://dx.doi.org/10.1016/j.revmed.2021.03.242.

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8

Ben Fadhel, N., H. Ammar, H. Ben Romdhane, K. Aouam, and N. Ben Fredj. "L’association du syndrome d’hypersensibilité médicamenteuse et la lymphohistiocytose hémophagocytaire : à propos de quatre cas." Revue Française d'Allergologie 61, no. 4 (May 2021): 285. http://dx.doi.org/10.1016/j.reval.2021.03.138.

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9

Spillane, David, and Jeffrey Wiseman. "Fever of Multiple Possible Origins." Canadian Journal of General Internal Medicine 16, no. 2 (June 21, 2021): 43–48. http://dx.doi.org/10.22374/cjgim.v16i2.461.

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A 60-year-old man presented with 1 week of fever despite broad-spectrum antibiotics for presumed pyelonephritis based on extended spectrum bacteriuria, recent bladder catheterization, and a negative search for other infections. He developed a maculopapular truncal rash, and pancytopenia with persistent fevers and worsening inflammatory markers despite modifying then stopping antibiotics. The non-specific clinical features at presentation and absence of hemophagocytosis on the initial bone marrow aspirate confounded multiple subspecialists and delayed the final diagnosis of hemophagocytic lymphohistiocytosis (HLH). Once this syndrome was elucidated, he responded well to dexamethasone and etoposide. An underlying diagnosis of systemic lupus erythematosus with aortic vasculitis was made, which in combination with pyelonephritis likely precipitated HLH. We summarize current concepts, pitfalls, and lessons learned in the diagnosis and management of HLH. RésuméUn homme de 60 ans se présente à l’hôpital à la suite d’une semaine de fièvre malgré la prise d’antibiotiques à large spectre pour traiter une pyélonéphrite soupçonnée, fondée sur une bactériurie à spectre étendu, un cathétérisme vésical récent et une recherche infructueuse d’autres infections. Il a développé une éruption cutanée maculopapulaire sur le tronc et une pancytopénie accompagnée d’une fièvre persistante et d’une augmentation des marqueurs de l’inflammation malgré la modification, puis l’arrêt des antibiotiques. Les manifestations cliniques non spécifiques à la présentation et l’absence d’hémophagocytose lors de la ponction médullaire initiale ont confondu de multiples surspécialistes et retardé le diagnostic définitif de lymphohistiocytose hémophagocytaire (LHH). Une fois que ce syndrome a été élucidé, le patient a bien répondu au traitement par la dexaméthasone et l’étoposide. Un diagnostic sous-jacent de lupus érythémateux systémique accompagné d’une vascularite de l’aorte a été posé qui, combiné à la pyélonéphrite, a probablement précipité la LHH. Nous résumons les concepts actuels, les pièges et les leçons apprises dans le diagnostic et la prise en charge de la LHH.
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10

Antonini, L., E. Klement, C. Cazorla, A. Merlet, A. Gourinat, M. Goujart, J. Colot, I. Mermoud, D. Raoult, and E. Descloux. "Description de 90 patients adultes atteints de lymphohistiocytose hémophagocytaire : place d’une nouvelle zoonose en milieu tropical." Médecine et Maladies Infectieuses 49, no. 4 (June 2019): S127. http://dx.doi.org/10.1016/j.medmal.2019.04.306.

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11

Zoubeidi, H., L. Baili, I. Rachdi, F. Daoud, Z. Aydi, S. Kochbati, B. Ben Dhaou, and F. Boussema. "Une lymphohistiocytose hémophagocytaire associée à une primo-infection à cytomégalovirus chez une patiente immunocompétente : une nouvelle observation." La Revue de Médecine Interne 36 (December 2015): A119. http://dx.doi.org/10.1016/j.revmed.2015.10.051.

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12

Pastissier, A., S. Humbert, J. Razanamahery, T. Soumagne, E. Daguindau, G. Piton, C. Chirouze, and N. Magy-Bertrand. "Lymphohistiocytose hémophagocytaire secondaire : comparaison de la présentation au diagnostic et de la survie suivant le caractère malin ou non de l’étiologie." La Revue de Médecine Interne 42 (June 2021): A50—A51. http://dx.doi.org/10.1016/j.revmed.2021.03.254.

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13

Tardieu, M., N. Janabi, S. Chabrier, and E. Haddad. "Production de NO, d'ions superoxydes et d'écosanoïdes par les cellules du système nerveux central. Un exemple clinique d'hyperactivation des macrophages: la lymphohistiocytose familiale." Archives de Pédiatrie 3, no. 9 (September 1996): 921. http://dx.doi.org/10.1016/0929-693x(96)87593-2.

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14

Ibrahim, Uroosa, Amina Saqib, Maryam Rehan, and Jean Paul Atallah. "ALK-Negative Anaplastic Large Cell Lymphoma Presenting as Disseminated Intravascular Coagulation and Hemophagocytic Lymphohistiocytosis: A Potentially Fatal Presentation." Case Reports in Hematology 2018 (2018): 1–3. http://dx.doi.org/10.1155/2018/3465351.

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Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disorder that can be familial in etiology or a result of infections, malignancy, and autoimmune or inflammatory disorders. Disseminated intravascular coagulation (DIC) is common in patients admitted to intensive care units and can confound and delay the diagnosis of HLH. We present a case of a 69-year-old female who presented with dyspnea and malaise. Her condition declined rapidly with laboratory parameters consistent with DIC. In addition, she had a ferritin of 32,522 ng/mL, low haptoglobin, and elevated LDH, and bone marrow biopsy showed hemophagocytic lymphohistiocytes. She was started on HLH-directed therapy, and later, a diagnosis of ALK-negative anaplastic large cell lymphoma was made on an excisional inguinal lymph node biopsy specimen. Our case emphasizes the importance of prompt recognition, diagnosis, and treatment of HLH while workup for a primary disorder is still being pursued.
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15

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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16

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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17

Coppola, Andrew, Chia Chey, Emma O’Donovan, and Monira Rahman. "A rare cause of acute liver failure due to haemophagocytic lymphohistiocytosis secondary to diffuse large B-cell lymphoma." JRSM Open 12, no. 3 (March 2021): 205427042098362. http://dx.doi.org/10.1177/2054270420983623.

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Acute liver failure is a life-threatening condition commonly caused by drug-induced hepatotoxicity or viral hepatitides. However, there are a number of rarer causes such as haemophagocytic lymphohistiocytosis. Haemophagocytic lymphohistiocytosis is a syndrome of uncontrolled immune cell activation, triggered by infection or malignancy, which carries a high mortality. Whilst mild to moderate liver injury is commonly seen with haemophagocytic lymphohistiocytosis, acute liver failure has rarely been reported in adults. We present a case of a 74-year-old man with acute liver failure secondary to haemophagocytic lymphohistiocytosis triggered by undiagnosed large B-cell lymphoma. Initially treated for biliary sepsis, there was a delay in the diagnosis of haemophagocytic lymphohistiocytosis and despite initiating chemotherapy, he died soon after. This case highlights the importance of considering haemophagocytic lymphohistiocytosis as a rare cause of acute liver failure, as given the life-threatening potential of haemophagocytic lymphohistiocytosis, a prompt diagnosis may allow early initiation of chemotherapy for any chance of survival.
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18

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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19

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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20

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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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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22

Stalder, Grégoire, Camillo Ribi, and Michel André Duchosal. "Les lymphohistiocytoses hémophagocytaires." Praxis 107, no. 16 (August 2018): 902–11. http://dx.doi.org/10.1024/1661-8157/a003045.

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Zusammenfassung. Die hämophagozytische Lymphohistiozytose (HLH) umfasst eine Gruppe seltener Krankheiten, welche mit einer schwerwiegenden Entzündungsreaktion durch ungezügelte Aktivierung des Immunsystems einhergehen. Für das Erscheinungsbild ist auch die Bezeichnung Makrophagenaktivierungssyndrom gebräuchlich. Man unterscheidet primäre und sekundäre Formen der HLH. Erstere treten bereits im Säuglingsalter auf und sind durch Mutationen im Bereich der zytotoxischen Lymphozytenfunktion bedingt. Die sekundären Formen werden durch Infekte, Autoimmunerkrankungen und bösartige Bluterkrankungen und Tumoren hervorgerufen oder sind idiopathisch. Die verschiedenen Formen der HLH manifestieren sich einheitlich mit hohem Fieber, einer Hepatosplenomegalie und Zytopenien. Ohne Behandlung kommt es zum Multiorganversagen mit meist tödlichem Verlauf. Die Diagnose beruht zurzeit auf der Zusammenstellung mehrerer klinischer und biologischer Merkmale. Die Behandlung basiert auf der Entfernung der Ursache, Unterstützung des Kreislaufes und der Organfunktion und der Verabreichung von Immunosuppressiva. Die primären HLH wurden intensiv erforscht und gut etablierte Therapieprotokolle liegen für pädiatrische Fälle vor. Es liegen nun auch mehrere retrospektive Studien über die sekundären HLH vor, welche vor allem Erwachsene betreffen und eine steigende Inzidenz verzeichnen. So werden nun neue Diagnosekriterien und moderne Behandlungsansätze vorgeschlagen, welche in diesem Artikel zusammengefasst sind.
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23

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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24

Patnayak, Rashmi, B. Manohar, PRavi Kumar, BSasikumar Reddy, and Shabbir Ali. "Haemophagocytic lymphohistiocytosis." Journal of Clinical and Scientific Research 3, no. 4 (2014): 261. http://dx.doi.org/10.15380/2277-5706.jcsr.13.064.

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25

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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26

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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27

Brown, Joanne, Meena Shingde, and Winny Varikatt. "Haemophagocytic lymphohistiocytosis." Pathology 48 (February 2016): S119. http://dx.doi.org/10.1016/j.pathol.2015.12.315.

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28

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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29

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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30

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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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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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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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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34

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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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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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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37

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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38

Khalidi, Hasan S., L. Jeffrey Medeiros, and Hector Battifora. "Lymphohistiocytoid Mesothelioma." American Journal of Clinical Pathology 113, no. 5 (May 1, 2000): 649–54. http://dx.doi.org/10.1309/7ecy-kn61-pp1m-52eb.

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39

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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40

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

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41

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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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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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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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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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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46

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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47

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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Abstract:
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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Jiang, Qian. "A Case of Hemophagocytic Lymphohistiocytosis Coexisting with Pulmonary Hypertension." International Journal of Clinical Case Reports and Reviews 2, no. 2 (May 5, 2020): 01–05. http://dx.doi.org/10.31579/2690-4861/010.

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Kılınçer Bozgül, Şükriye Miray, Ömer Emgin, Nur Akad Soyer, Özlem Ulusan, and Devrim Bozkurt. "Erişkin Bir Hastada Hemofagositik Lenfohistiyositoz ve Viseral Leyşmanyazis." Mikrobiyoloji Bulteni 54, no. 2 (April 15, 2020): 339–46. http://dx.doi.org/10.5578/mb.68974.

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