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Published in: International Journal of Legal Medicine 3/2016

01-05-2016 | Case Report

Rare cause of natural death in forensic setting: hemophagocytic syndrome

Authors: B. Ondruschka, J.-O. Habeck, C. Hädrich, J. Dreßler, R. Bayer

Published in: International Journal of Legal Medicine | Issue 3/2016

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Abstract

We report about the case of a sudden unexpected death of a 25-year-old male suffering from infectious disease. An autopsy was ordered with no final premortem diagnosis. Microscopic and microbiological examination revealed a pneumococcal bronchopneumonia and hemophagocytic lesions in the bone marrow. After integrating clinical and autopsy reports as well as additional postmortem investigations, the cause of death was found to be infectious-triggered hemophagocytic syndrome (HPS) with a final cytokine storm. This seems to be the first reported fatal case of a reactive form of HPS associated to Streptococcus pneumoniae to the best of our knowledge. HPS is a dangerous hyperinflammation with highly characteristic, but nonspecific, laboratory findings and symptoms. Autopsies in such cases must be carefully performed and include systematic tissue sampling done by an experienced pathologist.
Literature
1.
go back to reference De la Grandmaison GL, Clairand I, Durigon M (2001) Organ weight in 584 adult autopsies: new tables for caucasoid population. Forensic Sci Int 119:149–154CrossRef De la Grandmaison GL, Clairand I, Durigon M (2001) Organ weight in 584 adult autopsies: new tables for caucasoid population. Forensic Sci Int 119:149–154CrossRef
2.
go back to reference Scott RB, Robb-Smith AHT (1939) Histiocytic medullary reticulosis. Lancet 234:194–198CrossRef Scott RB, Robb-Smith AHT (1939) Histiocytic medullary reticulosis. Lancet 234:194–198CrossRef
3.
go back to reference Farquhar JW, Claireaux AE (1952) Familial haemophagocytic reticulosis. Arch Dis Child 27:519–525 Farquhar JW, Claireaux AE (1952) Familial haemophagocytic reticulosis. Arch Dis Child 27:519–525
4.
go back to reference Ansuini V, Rigante D, Esposito S (2013) Debate around infection-dependent hemophagocytic syndrome in paediatrics. BMC Infect Dis 13:15CrossRef Ansuini V, Rigante D, Esposito S (2013) Debate around infection-dependent hemophagocytic syndrome in paediatrics. BMC Infect Dis 13:15CrossRef
5.
go back to reference Shabbir M, Lucas J, Lazarchick J, Shirai K (2011) Secondary hemophagocytic syndrome in adults: a case series of 18 patients in a single institution and a review of literature. Heatmol Oncol 29:100–106 Shabbir M, Lucas J, Lazarchick J, Shirai K (2011) Secondary hemophagocytic syndrome in adults: a case series of 18 patients in a single institution and a review of literature. Heatmol Oncol 29:100–106
6.
go back to reference Rouphael NG, Talati NJ, Vaughan C, Cunningham K, Moreira R, Gould C (2007) Infections associated with haemophagocytic syndrome. Lancet Infect Dis 7:814–822CrossRef Rouphael NG, Talati NJ, Vaughan C, Cunningham K, Moreira R, Gould C (2007) Infections associated with haemophagocytic syndrome. Lancet Infect Dis 7:814–822CrossRef
7.
go back to reference Chute DJ, Rawley J, Cox J, Bready RJ (2013) Sudden unexpected death due to hemophagocytic lymphohistiocytosis syndrome. J Forensic Sci 58:1080–1084 Chute DJ, Rawley J, Cox J, Bready RJ (2013) Sudden unexpected death due to hemophagocytic lymphohistiocytosis syndrome. J Forensic Sci 58:1080–1084
8.
go back to reference Janka G (2006) Hämophagozytische syndrome. Monatsschr Kinderheilkd 154:1104–1109 Janka G (2006) Hämophagozytische syndrome. Monatsschr Kinderheilkd 154:1104–1109
9.
go back to reference Ravelli A (2002) Macrophage activation syndrome. Curr Opin Rheumatol 14:548–552CrossRef Ravelli A (2002) Macrophage activation syndrome. Curr Opin Rheumatol 14:548–552CrossRef
10.
go back to reference Janka GE (1983) Familial hemophagocytic lymphohistiocytosis. Eur J Pediatr 140:221–230 Janka GE (1983) Familial hemophagocytic lymphohistiocytosis. Eur J Pediatr 140:221–230
11.
go back to reference Erkut ZA, Ender E, Huitinga I, Swaab DF (2002) Cortisol is increased in postmortem cerebrospinal fluid of multiple sclerosis patients: relationship with cytokines and sepsis. Mult Scler 8:229–236CrossRef Erkut ZA, Ender E, Huitinga I, Swaab DF (2002) Cortisol is increased in postmortem cerebrospinal fluid of multiple sclerosis patients: relationship with cytokines and sepsis. Mult Scler 8:229–236CrossRef
12.
go back to reference InaiK NS, Iwasaki H, Naiki H (2014) Risk factor analysis for bone marrow histiocytic hyperplasia with hemophagocytosis: an autopsy study. Virchows Arch 465:109–118CrossRef InaiK NS, Iwasaki H, Naiki H (2014) Risk factor analysis for bone marrow histiocytic hyperplasia with hemophagocytosis: an autopsy study. Virchows Arch 465:109–118CrossRef
13.
go back to reference Henter JI, Horne A, Aricó M, Egeler RM, Filipovich AH, Imashuku S et al (2007) HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 48:124–131 Henter JI, Horne A, Aricó M, Egeler RM, Filipovich AH, Imashuku S et al (2007) HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 48:124–131
14.
go back to reference Janka GE, Schneider EM (2004) Modern management of children with haemophagocytic lymphohistiocytosis. Br J Haematol 124:4–14 Janka GE, Schneider EM (2004) Modern management of children with haemophagocytic lymphohistiocytosis. Br J Haematol 124:4–14
15.
go back to reference Rooms L, Fitzgerald N, McClain KL (2003) Hemophagocytic lymphohistiocytosis masquerading as child abuse: presentation of three cases and review of central nervous system findings in hemophagocytic lymphohistiocytosis. Pediatrics 111:e636–e640 Rooms L, Fitzgerald N, McClain KL (2003) Hemophagocytic lymphohistiocytosis masquerading as child abuse: presentation of three cases and review of central nervous system findings in hemophagocytic lymphohistiocytosis. Pediatrics 111:e636–e640
16.
go back to reference Frikke M, Hansen K (2004) Hemophagocytic lymphohistiocytosis (HLH). Pediatrics 114:1131–1132 Frikke M, Hansen K (2004) Hemophagocytic lymphohistiocytosis (HLH). Pediatrics 114:1131–1132
17.
go back to reference Arico M, Janka G, Fischer A, Henter JI, Blanche S, Elinder G et al (1996) Hemophagocytic lymphohistiocytosis. Report of 122 children from the International Registry. FHL Study Group of the Histiocyte Society. Leukemia 10:197–203 Arico M, Janka G, Fischer A, Henter JI, Blanche S, Elinder G et al (1996) Hemophagocytic lymphohistiocytosis. Report of 122 children from the International Registry. FHL Study Group of the Histiocyte Society. Leukemia 10:197–203
18.
go back to reference Reiner AP, Spivak JL (1988) Hematophagic histiocytosis. A report of 23 new patients and a review of the literature. Medicine (Baltimore) 67:369–388 Reiner AP, Spivak JL (1988) Hematophagic histiocytosis. A report of 23 new patients and a review of the literature. Medicine (Baltimore) 67:369–388
19.
go back to reference Chen CJ, Huang YC, Jaing TH, Hung U, Yang CP, Chang LY, Lin TY (2004) Hemophagocytic syndrome: a review of 18 pediatric cases. J Microbiol Immunol Infect 37:157–163PubMed Chen CJ, Huang YC, Jaing TH, Hung U, Yang CP, Chang LY, Lin TY (2004) Hemophagocytic syndrome: a review of 18 pediatric cases. J Microbiol Immunol Infect 37:157–163PubMed
20.
go back to reference Sun HY, Chen MY, Fang CT, Hsieh SM, Hung CC, Chang SC (2004) Hemophagocytic lymphohistiocytosis: an unusual initial presentation of acute HIV infection. J Acquir Immune Defic Syndr 37:1539–1540 Sun HY, Chen MY, Fang CT, Hsieh SM, Hung CC, Chang SC (2004) Hemophagocytic lymphohistiocytosis: an unusual initial presentation of acute HIV infection. J Acquir Immune Defic Syndr 37:1539–1540
21.
go back to reference Janka G, Imashuku S, Elinder G, Schneider M, Henter JI (1998) Infection- and malignancy-associated hemophagocytic syndromes. Secondary hemophagocytic lymphohistiocytosis. Hematol Oncol Clin North Am Apr 12:435–444 Janka G, Imashuku S, Elinder G, Schneider M, Henter JI (1998) Infection- and malignancy-associated hemophagocytic syndromes. Secondary hemophagocytic lymphohistiocytosis. Hematol Oncol Clin North Am Apr 12:435–444
22.
go back to reference Canna SW, Behrens EM (2012) Making sense of the cytokine storm: a conceptual framework for understanding, diagnosing and treating hemophagocytic syndromes. Pediatr Clin North Am 59:329–344CrossRef Canna SW, Behrens EM (2012) Making sense of the cytokine storm: a conceptual framework for understanding, diagnosing and treating hemophagocytic syndromes. Pediatr Clin North Am 59:329–344CrossRef
23.
go back to reference Li J, Wang Q, Zheng W, Ma J, Zhan W, Wang W et al (2014) Hemophagocytic lymphohistiocytosis. Clinical analysis of 103 adult patients. Medicine 93:100–105 Li J, Wang Q, Zheng W, Ma J, Zhan W, Wang W et al (2014) Hemophagocytic lymphohistiocytosis. Clinical analysis of 103 adult patients. Medicine 93:100–105
24.
go back to reference Sung L, Weitzmann SS, Petric M, King SM (2001) The role of infections in primary hemophagocytic lymphohistiocytosis: a case series and review of the literature. Clin Infect Dis 33:1644–1648 Sung L, Weitzmann SS, Petric M, King SM (2001) The role of infections in primary hemophagocytic lymphohistiocytosis: a case series and review of the literature. Clin Infect Dis 33:1644–1648
25.
go back to reference Dubuc CE, Ecenarro MU, Aguirre NE, Otano JB (2014) Macrophage activation syndrome as a severe manifestation of adult’s Still’s disease. Hemophagocytic cells in ascites. Reumatol Clin 420–421. Dubuc CE, Ecenarro MU, Aguirre NE, Otano JB (2014) Macrophage activation syndrome as a severe manifestation of adult’s Still’s disease. Hemophagocytic cells in ascites. Reumatol Clin 420–421.
26.
go back to reference Bell MD, Wright RK (1992) Fatal virus-associated hemophagocytic syndrome in a young adult producing nontraumatic splenic rupture. J Forensic Sci 37:1407–1417PubMed Bell MD, Wright RK (1992) Fatal virus-associated hemophagocytic syndrome in a young adult producing nontraumatic splenic rupture. J Forensic Sci 37:1407–1417PubMed
27.
go back to reference Clement R, Jouan H, Le Gall F, Rodat O (2006) Macrophage activation syndrome: an autopsy case of sudden death. J Clin Forensic Med 13:356–360CrossRef Clement R, Jouan H, Le Gall F, Rodat O (2006) Macrophage activation syndrome: an autopsy case of sudden death. J Clin Forensic Med 13:356–360CrossRef
28.
go back to reference Roll P, Beham A, Beham-Schmid C (2009) Post-mortem histopathological investigations of the bone marrow in forensic medicine: an important issue for both the forensic and clinical pathologist. Forensic Sci Int 186:e17–e20CrossRef Roll P, Beham A, Beham-Schmid C (2009) Post-mortem histopathological investigations of the bone marrow in forensic medicine: an important issue for both the forensic and clinical pathologist. Forensic Sci Int 186:e17–e20CrossRef
29.
go back to reference Tattoli L, Tsokos M, Sautter J, Anagnostopoulos J, Maselli E, Ingravallo G et al (2014) Postmortem bone marrow analysis in forensic science: study of 73 cases and review of the literature. Forensic Sci Int 234:72–78CrossRef Tattoli L, Tsokos M, Sautter J, Anagnostopoulos J, Maselli E, Ingravallo G et al (2014) Postmortem bone marrow analysis in forensic science: study of 73 cases and review of the literature. Forensic Sci Int 234:72–78CrossRef
Metadata
Title
Rare cause of natural death in forensic setting: hemophagocytic syndrome
Authors
B. Ondruschka
J.-O. Habeck
C. Hädrich
J. Dreßler
R. Bayer
Publication date
01-05-2016
Publisher
Springer Berlin Heidelberg
Published in
International Journal of Legal Medicine / Issue 3/2016
Print ISSN: 0937-9827
Electronic ISSN: 1437-1596
DOI
https://doi.org/10.1007/s00414-015-1305-0

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