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ISSN 0185-3252 (Print)
Revista de la Asociación Médica del Centro Médico ABC

2002, Number 4

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An Med Asoc Med Hosp ABC 2002; 47 (4)

Hemophagocytic syndrome associated with a hidden non-Hodgkin’s lymphoma: A case report of an adolescent.

Frenkel-Salamón M, Bolea-Murga V, Villarreal-López-Guerra G, Durán-Padilla MA

Language: Spanish
References: 21
Page: 223-227
PDF size: 157.73 Kb.


ABSTRACT

Introduction: Hemophagocytic Syndrome (HPS) is an infrequent pathological disorder in which histiocyte proliferation is associated to the phagocytosis of hematopoietic elements, producing heterogeneous clinical and paraclinical data. We describe the case of a 15 year old adolescent who presented an HPS reactive to neoplasic disease and Epstein-Barr infection. Clinical case: 15 year old female adolescent exhibiting persistent fever, malaise, anorexia, weight loss, hepatosplenomegaly, pancytopenia, hypofibrinogenemia, and hemophagocytosis in the bone marrow aspirate. Specific therapy was undertaken. Developed progressive neurological deterioration and death. The necropsy report described the presence of peripheral T-cell non-Hodgkin’s lymphoma in the mediastinal, peripancreatic, and periaortic lymph nodes; antiEpstein-Barr IgG; and reactive HPS. Conclusions: HPS is associated to both neoplasias and infections, particularly lymphomas and Epstein-Barr virus infection. Initial treatment is carried out by observation and support measures in the Intensive Pediatric Care Unit. Its prognosis is poor, in patients with neoplasic diseases associated to Epstein-Barr virus infection, the mortality is high.


REFERENCES

  1. Herrero-Hernández A, Ramírez-Jiménez S, García-Martín F, Martinez-Valverde A. Síndromes hemofagocíticos. An Esp Pediatr 1998; 49: 230-236.

  2. Chan JK, Ng CS, Law CK, Ng WF, Wong KF. Reactive hemophagocytic syndrome: A study of 7 fatal cases. Pathology 1987; 19: 43-50.

  3. Ladisch S. Histiocytosis syndromes of childhood. In: Nelson WE, Behrman RE, Kliegman RM, Arvin AN (eds). Nelson textbook of pediatrics. 16th ed. Philadelphia, PA: WB Saunders, 2000; 1570-1572.

  4. Komp DM. Historical perspectives of Langerhans cell histiocytosis. Hematol Oncol Clin North Am 1987; 1: 9-21.

  5. Risdall RJ, McKenna RW, Nesbit ME et al. Virus-associated hemophagocytic syndrome, a benign histiocytic proliferation distinct from malignant histiocytosis. Cancer 1979; 44: 993-1002.

  6. Janka G Imashuku S, Elinder G, Schneider M, Hunter JI. Infection an malignancy associated hemophagocytic syndromes. Hematol Oncol Clin North Am 1998; 12: 435-443.

  7. Siberry GK. Growth charts. En: Siberry GK, Iannonne E, (eds). The Harriet Lane Handbook. 15th ed. St Louis, Missouri: Mosby, 2000; 284-291.

  8. Ladisch S, Jaffe E. The histiocytoses. En: Pizzo PA, Poplack DG (eds). Principles and practice of pediatric oncology. 3rd ed. Philadelphia, PA: Lippincott-Raven Publishers, 1997; 615-631.

  9. Blanche S. Lymphohistiocytose Familiale et autres syndromes d’activation macrophagique. Edition Techniques-Encycl. Méd Chir (Paris-France), Pédiatrie, 4-082-J-40, 1995; 2.

  10. Baumann MA, Libnoch JA. Hemophagocytosis. JAMA 1984; 252: 1340.

  11. Astigarra-Aguirre I, Fernández-Teijeiro A, García-Perez N, Piñan-Francés MA, Navajas-Gutiérrez. Síndrome hemofagocítico en la infancia: a propósito de 4 casos. An Esp Pediatr 1998; 49: 289-293.

  12. Eakle JF, Bressoud PF. Hemophagocytic syndrome following an Epstein-Barr virus infection: A case report and literature review. Ky Med Assoc 2000; 98: 161-165.

  13. Campbell JK, Mitchell CA. Immune thrombocytopenia in association with acute lymphoblastic leukaemia and haemophagocytic syndrome. Eur J Haematol 1993; 51: 259-261.

  14. Nakamoto T, Ogawa S, Mano H, Hirai H, Yazaki Y. Haemophagocytic syndrome associated with non-Hodgkin’s lymphoma of B-cell type. Am J Hematol 1994; 47: 335-336.

  15. Linn YC, Tien SL, Lim LC, Lee LH, Teoh G, Goh YT et al. Haemophagocytosis in bone marrow aspirate. A review of clinical course of 10 cases. Acta Haematol 1995; 94: 182-191.

  16. Henter J-I, Elinder G, Öst A. The FHL study group of the Histiocyte Society. Diagnostic guidelines for hemophagocytic lymphohistiocytosis. Semin Oncol 1991; 18: 29-33.

  17. Henter JI, Aricò M Egeler RM, Elinder G, Fabra BE, Filipovich AH et al. HLH-94: A treatment protocol for Hemophagocytic lymphohistiocytosis. Med Pediatr Oncol 1997; 28: 342-347.

  18. Imashuku S, Kuriyama K, Teramura T, Ishii E, Kinugawa N, Kato M et al. Requirement for etoposide in the treatment of Epstein-Barr virus-associated hemophagocytic limphohistiocytosis. J Clin Oncol 2001; 19: 2665-2673.

  19. Imashuku S. Advances in the management of hemophagocytic limphohistiocytosis. Int J Hematol 2000; 72: 1-11.

  20. Gagnaine MH, Galambrun C, Stephan JL. Hemophagocytic syndrome: A misleading complication of visceral leishmaniasis in children – A series of 12 cases. Pediatrics 2000; 106: e58.

  21. Imashuku S, Hibi S, Ohara T, Iwai A, Sako M, Kato M et al. Effective control of Epstein-Barr virus-related hemophagocytic limphohistiocytosis with immunochemotherapy. Blood 1999; 93: 1869-1874.




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