Secondary Hemophagocytic Lymphohistiocytosis
Keywords:
Hemophagocytic lymphohistiocytosis, Activated macrophage, immune activation
Abstract
Introduction : Hemophagocytic lymphohistiocytosis (HLH) is a rare hematological disorder which may be fatal due to uncontrolled activation of immune system. It is characterized by hemophagocytosis in bone marrow, liver or lymph nodes along with presence of fever, pancytopenia, splenomegaly. It has been associated with bacterial, viral, fungal and parasitic infection. The study aims to explore the utility of bone marrow aspiration in patients with pancytopenia and bicytopenia which supports the diagnosis in a scenario where there is clinical suspicion of HLH. Material and Methods – prospective study carried out in department of pathology from August 2018 to July 2019. We studied 88 cases of pancytopenia and bicytopenia. All relevant clinical history and investigations were collected. Bone marrow aspiration done and smears were studied in all the cases. Result – Out of 88 cases studied 28 patients were having bicytopenia and 60 were having pancytopenia with male to female ratio 13 :9.Out of these 88 patients we reported 3 patients as having HLH who fulfill the criteria according to Revised Diagnostic Guidelines for hemophagocytic lymphohistiocytosis (HLH). Conclusion :-An early diagnosis and treatment is the key to decrease the mortality caused by this dreadful disease and if left undiagnosed and undertreated, Hemophagocytic lymphohistiocytosis can be rapidly progressive and potentially fatal.References
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4. .Mancebo E, Allende LM, Guzmán M, Paz Artal E, Gil J, Urrea Moreno R, et al. Familial hemophagocytic lymphohistiocytosis in an adult patient homozygous for A91V in the perforin gene, with tuberculosis infection. Haematologica 2006;91:1257 60.
5. Otrock ZK, Eby CS: Clinical characteristics, prognostic factors, and outcomes of adult patients with hemophagocytic lymphohistiocytosis. Am J Hematol. 2014, 90:220- 224. 10.1002/ajh.23911
6. Janka GE. Familial and acquired hemophagocytic lymphohistiocytosis. Annu Rev Med. 2012
7. Chandra P, Chaudhery SA, Rosner F, Kagen M. Transient histiocytosis with striking phagocytosis of platelets, leukocytes, and erythrocytes. Arch Int Med 1975;135:989 91.
8. Henter JI, Horne A, Arcio M, et al, HLH 2004,Diagnostic and therapeutic guidelines for Hemophagocytic Lymphohistiocytosis. Pediatric Blood Cancer 2007;48:124-131.
9. Park HS, Kim DY, Lee JH, Lee JH, Kim SD, Park YH, et al. Clinical features of adult patients with secondary hemophagocytic lymphohistiocytosis from causes other than lymphoma: An analysis of treatment outcome and prognostic factors. Ann Hematol 2012;91:897 904.
10. Imashuku S, Kuriyama K, Sakai R, Nakao Y, Masuda S, Yasuda N, et al. Treatment of Epstein Barr virus associated hemophagocytic lymphohistiocytosis (EBV HLH) in young adults: A report from the HLH study center. Med Pediatr Oncol 2003;41:103 9
11. Hori M, Yoshida R, Aoyama I, Ichida S. Case of tuberculosis associated hemophagocytic syndrome in a hemodialysis patient under steroid therapy. Nippon Jinzo Gakkai Shi 2009;51:1091 5
12.Henter JI, Horne A, Aricó M, Egeler RM, Filipovich AH, Imashuku S, et al. HLH 2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007;48:124 31.
13. Ho C, Yao X, Tian L, Li FY, Podoltsev N, Xu ML. Marrow assessment of hemophagocytic lymphohistiocytosis demonstrates poor correlation with disease probability. Am J Clin Pathol 2014;141:62 71.
2. Lin TF, Ferlic Stark LL, Allen CE, Kozinetz CA, McClain KL. Rate of decline of ferritin in patients with hemophagocytic lymphohistiocytosis as a prognostic variable for mortality. Pediatr Blood Cancer 2011;56:154 5.
3. Nikiforow S, Berliner N: The unique aspects of presentation and diagnosis of hemophagocytic lymphohistiocytosis in adults. Hematology Am Soc Hematol Educ Program. 2015, 2015:183- 189. 10.1182/asheducation-2015.1.183
4. .Mancebo E, Allende LM, Guzmán M, Paz Artal E, Gil J, Urrea Moreno R, et al. Familial hemophagocytic lymphohistiocytosis in an adult patient homozygous for A91V in the perforin gene, with tuberculosis infection. Haematologica 2006;91:1257 60.
5. Otrock ZK, Eby CS: Clinical characteristics, prognostic factors, and outcomes of adult patients with hemophagocytic lymphohistiocytosis. Am J Hematol. 2014, 90:220- 224. 10.1002/ajh.23911
6. Janka GE. Familial and acquired hemophagocytic lymphohistiocytosis. Annu Rev Med. 2012
7. Chandra P, Chaudhery SA, Rosner F, Kagen M. Transient histiocytosis with striking phagocytosis of platelets, leukocytes, and erythrocytes. Arch Int Med 1975;135:989 91.
8. Henter JI, Horne A, Arcio M, et al, HLH 2004,Diagnostic and therapeutic guidelines for Hemophagocytic Lymphohistiocytosis. Pediatric Blood Cancer 2007;48:124-131.
9. Park HS, Kim DY, Lee JH, Lee JH, Kim SD, Park YH, et al. Clinical features of adult patients with secondary hemophagocytic lymphohistiocytosis from causes other than lymphoma: An analysis of treatment outcome and prognostic factors. Ann Hematol 2012;91:897 904.
10. Imashuku S, Kuriyama K, Sakai R, Nakao Y, Masuda S, Yasuda N, et al. Treatment of Epstein Barr virus associated hemophagocytic lymphohistiocytosis (EBV HLH) in young adults: A report from the HLH study center. Med Pediatr Oncol 2003;41:103 9
11. Hori M, Yoshida R, Aoyama I, Ichida S. Case of tuberculosis associated hemophagocytic syndrome in a hemodialysis patient under steroid therapy. Nippon Jinzo Gakkai Shi 2009;51:1091 5
12.Henter JI, Horne A, Aricó M, Egeler RM, Filipovich AH, Imashuku S, et al. HLH 2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007;48:124 31.
13. Ho C, Yao X, Tian L, Li FY, Podoltsev N, Xu ML. Marrow assessment of hemophagocytic lymphohistiocytosis demonstrates poor correlation with disease probability. Am J Clin Pathol 2014;141:62 71.
Published
2020-01-04
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