Difficult Diagnosis: A Case of Hemophagocytic Lymphohistocytosis in an Infant
K. V. Pshenisnov,
Yu. S. Аleksandrovich,
M. A. Udaltsov,
T. A. Аnokhin,
V. A. Kaziakhmedov,
L. M. Pochinyaeva,
V. E. Ironosov https://doi.org/10.21292/2078-5658-2022-19-1-91-98
Abstract
Hemophagocytic lymphohistocytosis is an extremely rare disease requiring early diagnosis and specific treatment.
The objective: to demonstrate the clinical case of hemophagocytic lymphohistocytosis in an infant. The course of the disease in a 6-month-old child requiring treatment in the intensive care unit was retrospectively analyzed. The article presents the procedure of differential diagnosis of sepsis and hemophagocytic lymphohistocytosis in children, special attention is paid to the diagnostic criteria, and the clinical and laboratory differences of these diseases are described.
Conclusion. The presence of leukopenia, neutropenia, thrombocytopenia, hypoalbuminemia and hypocoagulation in an infant with persistent fever is the basis for a thorough differential diagnosis in order to eliminate severe systemic diseases that manifest as childhood infections and sepsis.
About the Authors
K. V. Pshenisnov
St. Petersburg State Pediatric Medical University
Russian Federation
Russian Federation
Konstantin V. Pshenisnov, Doctor of Medical Sciences, Associate Professor of Anesthesiology, Intensive Care and Emergency Pediatrics Department within Post-Graduate and Continuing Professional Development Faculty.
2, Litovskaya St., St. Petersburg, 194100.
Yu. S. Аleksandrovich
St. Petersburg State Pediatric Medical University
Russian Federation
Russian Federation
Yury S. Aleksandrovich, Doctor of Medical Sciences, Professor, Pro-Rector for Post-Graduate and Continuing Professional Education and Regional Public Health Development, Head of Anesthesiology, Intensive Care and Emergency Pediatrics Department within Post-Graduate and Continuing Professional Development Faculty.
2, Litovskaya St., St. Petersburg, 194100.
M. A. Udaltsov
N. F. Filatov Children City Hospital no. 5
Russian Federation
Russian Federation
Maksim A. Udaltsov, Anesthesiologist and Emergency Physician of Anesthesiologym and Intensive Care Department.
134, Bukharestskaya St., St. Petersburg, 192289.
T. A. Аnokhin
N. F. Filatov Children City Hospital no. 5
Russian Federation
Russian Federation
Taras A. Anokhin, Anesthesiologist and Emergency Physician of Anesthesiology and Intensive Care Department.
134, Bukharestskaya St., St. Petersburg, 192289.
V. A. Kaziakhmedov
St. Petersburg State Pediatric Medical University; N. F. Filatov Children City Hospital no. 5
Russian Federation
Russian Federation
Vitaliy A. Kaziakhmedov, Candidate of Medical Sciences, Associate Professor of Anesthesiology, Intensive Care and Emergency Pediatrics Department within Post-Graduate and Continuing Professional Development Faculty.
2, Litovskaya St., St. Petersburg, 194100.
L. M. Pochinyaeva
N. F. Filatov Children City Hospital no. 5
Russian Federation
Russian Federation
Lyubov M. Pochinyaeva, Deputy Head Doctor for Treatment.
134, Bukharestskaya St., St. Petersburg, 192289.
V. E. Ironosov
St. Petersburg State Pediatric Medical University
Russian Federation
Russian Federation
Vyacheslav E. Ironosov, Candidate of Medical Sciences, Associate Professor of Anesthesiology, Intensive Care and Emergency Pediatrics Department within Post-Graduate and Continuing Professional Development Faculty
2, Litovskaya St., St. Petersburg, 194100.
References
1. Аleksandrovich Yu.S., Pshenisnov K.V., Felker E.Yu. et al. Urea cycle defects causing acute cerebral failure in children: case report. Vestnik Intensivnoy Terapii, 2017, vol. 1, pp. 73‒79.(In Russ.)
2. Ivanov D.O., Pshenisnov K.V., Аleksandrovich Yu.S. Features of intensive care of congenital heart malformations in newborns. Pediatriya, 2020, vol. 99, no. 3, C. 200-207. (In Russ.) doi: 10.24110/0031-403X-2020-99-3-200-207.
3. Lekmanov А.U., Mironov P.I., Аleksandrovich Yu.S. et al. Sepsis in children: federal clinical guidelines (draft). Rossiyskiy. Vestnik Detskoy Khirurgii, Anesteziologii I Reanimatologii, 2021, vol. 11, no. 2, pp. 241-292. (In Russ.) doi: https://doi.org/10.17816/psaic969.
4. Alvi S., Naqvi A. Advances in the diagnosis and management of haemophagocytic lymphohistiocytosis: a review of literature. LymphoSign J., 2018, vol. 5, pp. 1‒15. doi:10.14785/lymphosign-2017-0010.
5. Arico M., Janka G., Fischer A. et al. Hemophagocytic lymphohistiocytosis. Report of 122 children from the International Registry. FHL Study Group of the Histiocyte Society. Leukemia, 1996, vol. 10, no. 2, pp. 197‒203.
6. Cleves D., Lotero V., Medina D. et al. Pediatric hemophagocytic lymphohistiocytosis: A rarely diagnosed entity in a developing country. BMC Pediatr., 2021, vol. 21, no. 1, pp. 411. doi: 10.1186/s12887-021-02879-7.
7. George M. Hemophagocytic lymphohistiocytosis: review of etiologies and management. J. Blood Med., 2014, vol. 5, pp. 69‒86. doi:10.2147/JBM.S46255.
8. Gupta A.A., Tyrrell P., Valani R. et al. Experience with hemophagocytic lymphohistiocytosis/macrophage activation syndrome at a single institution. J. Pediatr. Hematol. Oncol., 2009, vol. 31, no. 2, pp. 81‒84. doi: 10.1097/MPH.0b013e3181923cb4.
9. Henter J.I., Horne A., Aricó M. et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr. Blood Cancer, 2007, vol. 48, no. 2, pp. 124‒131. doi: 10.1002/pbc.21039.
10. Machowicz R., Janka G., Wiktor-Jedrzejczak W. Similar but not the same: Differential diagnosis of HLH and sepsis. Crit. Rev. Oncol. Hematol., 2017, vol. 114, pp. 1‒12. doi: 10.1016/j.critrevonc.2017.03.023.
11. Madkaikar M., Shabrish S., Desai M. Current updates on classification, diagnosis and treatment of hemophagocytic lymphohistiocytosis (HLH). Indian. J. Pediatr., 2016, vol. 83, no. 5, pp. 434–443. doi: 10.1007/s12098-016-2037-y.
12. Sung L., King S.M., Carcao M. et al. Adverse outcomes in primary hemophagocytic lymphohistiocytosis. J. Pediatr. Hematol. Oncol., 2002, vol. 24, no. 7, pp. 550‒554. doi: 10.1097/00043426-200210000-00011.
Review
For citations:
Pshenisnov K.V., Аleksandrovich Yu.S., Udaltsov M.A., Аnokhin T.A., Kaziakhmedov V.A., Pochinyaeva L.M., Ironosov V.E. Difficult Diagnosis: A Case of Hemophagocytic Lymphohistocytosis in an Infant. Messenger of ANESTHESIOLOGY AND RESUSCITATION. 2022;19(1):91-98. (In Russ.) https://doi.org/10.21292/2078-5658-2022-19-1-91-98
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