2002, Number 4
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Rev Med Hosp Gen Mex 2002; 65 (4)
Reactive hemophagocytic syndrome. A post mortem study of 24 pediatric cases
Morales-Ferrer G, Durán-Padillam MA, Córdova RS
Language: Spanish
References: 23
Page: 207-212
PDF size: 104.72 Kb.
ABSTRACT
Hemophagocytic syndrome is an heterogeneous disease, clinically it presents with fever, anemia pancytopenia or multiple organ failure. Histologically it afects the phagocytic system with the presence of hemophagocytosis in the spleen, liver, lymph nodes and bone marrow. It afects children (1–2/1.000.000) with survival rates of 60%. 24 cases of hemophagocytic syndrome were selected from pediatric pathology autopsy reports and the morphologic changes were studied, concomitant diseases four were acute lymphoblastic leukemia (ALL) 33%, acute myeloid leukemia (AML) 8%, myelodisplastic syndrome (MS) 4%, non–Hodgkin lymphoma 25%. Infectious agents were also four:
Salmonella typhi 4%,
Staphylococcus epidermidis 8%,
Klebsiella 4%,
Candida albicans 4%, Epstein-Barr virus 4% and hepatitis B virus 4%. Intense histiocytosis associated with leukemias and lymphomas was prominent in lymph node and bone marrow fatal cases were related to Epstein-Barr virus (+) T–cell lymphoma, acute lymphocytic leukemia and hepatitis B. We conclude that pathologic examination of lymph nodes and bone marrow is a useful tool for the diagnosis of hemophagocytic syndrome.
REFERENCES
Risdall JR, McKenna WR, Nesbit EM, Krivit W, Balfour Jr HH, Simmons LR et al. Virus- associated hemophagocytic syndrome. Cancer 1979; 44: 993-1002.
Aouad A, Dan EM, Alangade JG. Diagnosis: Reactive hemophagocytic syndrome caused by CMV infection. Clin Infect Dis 1998; 26: 1295.
Wong FK, Chan CKJ, Ha YS, Wong WH. Reactive hemophagocytic syndrome in childhood- frequent occurrence of atypical mononuclear cells. Hematol Oncol 1994; 12: 67-74.
Herrero HA, Ramíres JS, García ME, Martínez VA. Síndromes hemofagocíticos. An Esp Pediatr 1998; 49: 230-236.
Osugi Y, Hara J, Tagawa S, Takai K, Hosoi G, Matsuda Y, Fujisaki H et al. Cytokine production regulating Th1 and Th2 cytokines in hemophagocytic lymphohistiocytosis. Blood 1997; 11: 4100-4103.
Cline MJ. Histiocytes and histiocytosis. Blood 1994; 84: 2840-53.
Frenkel SM, Bolea MV, Durán Padilla MA. Síndrome hemofagocítico en pediatría. An Med Asoc Med Hosp ABC 2001; 46: 137-141.
Manivel RC, Jessurum SJ, Rodríguez MA. Histiocitosis reactiva hemofagocítica. Patología 1983; 21: 175-186.
Janka G, Imashuku S, Elinder G, Schneider M, Henter J. Infection and malignancy associated hemophagocytic syndromes. Hematol Oncol Clin North Am 1998; 12: 67-74.
García JDD, Guijarro HC, Abarca CM, Guerra Vales JM, Histiocitosis reactiva, hemofagocitosis histiocítica y síndromes hemofagocíticos. Rev Clin Esp 1990; 186: 193-194.
Favara EB. Hemophagocytic limphohistiocytosis: A hemophagocytic syndrome. Semin Diagnosis Pathol 1992; 9: 63-74.
Kerguenec C, Hillaire S, Moliné V, Gardin C, Degott C, Erlinger S et al. Hepatic manifestations of hemophagocytic syndrome: A study of 30 cases. Am J Gastroenterol 2001; 96: 852-857.
Allory A, Challine D, Haioun C, Bergman CC, Delfau LHM, Boucher E. Bone marrow involvement in lymphomas with hemophagocytic syndrome at presentation. Am J Surg Pathol 2001; 25: 865-874.
Takasaki N, Kaneko Y, Maseki N, Sakura M, Shimamura K, Takayama S. Hemophagocytic syndrome complicating T- cell acute lymphoblastic leukemia with a novel t(11; 14) (p 15; q11) chromosome translocation. Cancer 1987; 59: 424-428.
Jaffe SE, Costa J, Fauci SA, Cossman J, Tsokos M. Malignant lymphoma and erythrophagocytosis simulating malignant histiocytosis. Am J Med 1983; 75: 741-49.
16 Chan CKJ, Law KC, Wong FK. Reactive hemophagocytic syndrome: A study of 7 fatal cases. Pathology 1987; 19: 43-50.
Astigarra AI, Fernandez TA, García PN , Piña FMA, Navajas GA. Síndrome hemofagocítico en la infancia: A propósito de 4 casos. An Esp Pediatr 1998; 49: 289–293.
Falini B, Pileri S, De Solas I, Martelli MF, Mason DY, Delsol G et al. Peripheral T cell Lymphoma Associated with Hemophagocytic Syndrome. Blood 1990; 75: 434–444.
19 Mroczek CE, Weisenburger DD, Grierson LH, Markin R, Purtilo T D. Fatal infectious mononucleosis and virus associated hemophagocytic syndrome. Arch Pathol Lab Med 1987; 111: 530-535.
Fisman N D. Hemophagocytic syndromes and infection. Emerg Infect Dis 2000; 6: 601-608.
Imashuku S, Hibi S, Ohara T, Iwai A, Sako M, Kato M et al. Effective control of Epstein- Barr virus- related hemophagocytic lymphohistiocytosis with immunochemotherapy. Blood 1999; 93: 1869-1874.
Kikuta H, Sakiyama Y, Matsymoto S, Oh-Ishi T, Nakano T, Nagashima T et al. Fatal Epstein-Barr virus associated hemophagocytic syndrome. Blood 1993; 82: 3259-3264.
Imashuku S, Hibi S, Todo S. Hemophagocytic lymphohistiocytosis in infancy and childhood. J Pediatr 1997; 130: 352-357.