A 46-year-old Dominican man, known to have HIV, presented with constitutional symptoms of two week's duration. The patient was found to have cytopenias, significantly elevated ferritin level and lymphadenopathy. Biopsies and laboratory studies met the criteria for hemophagocytic lymphohistiocytosis (HLH). A concomitant diagnosis of histoplasmosis was confirmed as the trigger for HLH and treatment resulted in clinical improvement and resolution of symptoms.
HenterJIet al.
HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer2007;
48: 124–131.
2.
OtrockZKEbyCS.Clinical characteristics, prognostic factors, and outcomes of adult patients with hemophagocytic lymphohistiocytosis. Am J Hematol2015;
90: 220–224.
3.
GeorgeMR.Hemophagocytic lymphohistiocytosis: review of etiologies and management. J Blood Med2014;
5: 69–86.
4.
ChandrakasanSFilipovichAH.Hemophagocytic lymphohistiocytosis: advances in pathophysiology, diagnosis, and treatment. J Pediatr2013;
163: 1253–1259.
5.
JankaGE.Familial and acquired hemophagocytic lymphohistiocytosis. Annu Rev Med2012;
63: 233–246.
6.
FilipovichAMcClainKGromA.Histiocytic disorders: recent insights into pathophysiology and practical guidelines. Biol Blood Marrow Transplant2010;
16: S82–S89.
7.
ImashukuSet al.
Treatment of Epstein–Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) in young adults: a report from the HLH study center. Med Pediatr Oncol2003;
41: 103–109.
RosadoFGKimAS.Hemophagocytic lymphohistoiocytosis: update on diagnosis and pathogenesis. Am J Clin Pathol . 2015;
139: 713–727.
10.
WeitzmanS.Approach to hemophagocytic syndromes. Hematology Am Soc Hematol Educ Program2011; 1: 178–183.
11.
TownsendJLet al.
Histoplasmosis-induced hemophagocytic syndrome: a case series and review of the literature. Open Forum Infect Dis2015;
2: 55.
12.
CasteilliAAet al.
Hemophagocytic lymphohistiocytosis secondary to human immunodeficiency virus-associated histoplasmosis. Open Forum Infect Dis2015;
2: 140.
13.
JankaG.Hemophagocytic lymphohistiocytosis: when the immune system runs amok. Klin Padiatr2009;
221: 278–285.
14.
GromAA.Natural killer cell dysfunction: a common pathway in systemic-onset juvenile arthritis, macrophage activation syndrome, and hemophagocytic lymphohistiocytosis?Arthritis Rheum2004;
50: 689–698.
15.
JordanMBet al.
An Animal Model of Hemophagocytic Lymphohistiocytosis (HLH): CD8+ T cells and Interferon Gamma are Essential for the Disorder. Blood2004;
104: 735–743.
16.
TincatiCet al.
Invariant natural killer T (iNKT) cells in HAART-treated, HIV-positive patients with bone and cardiovascular impairment. PLoS ONE2014;
9: e110287.
17.
ParasaVRet al.
Interleukins 15 and 12 in combination expand the selective loss of natural killer T cells in HIV infection in vitro. Clin Exp Med. 2014;
15: 205–213.
18.
NaluyimaPet al.
Impaired natural killer cell responses are associated with loss of the highly activated NKG2A(+)CD57(+)CD56(dim) subset in HIV-1 subtype D infection in Uganda. AIDS2014;
28: 1273–1278.
19.
MerinoAMet al.
KIR2DS4 promotes HIV-1 pathogenesis: new evidence from analyses of immunogenetic data and natural killer cell function. PLoS ONE2014;
9: e99353.
20.
DillonSMet al.
The natural killer cell interferon-gamma response to bacteria is diminished in untreated HIV-1 infection and defects persist despite viral suppression. J Acquir Immune Defic Syndr2014;
65: 259–267.
21.
ParkK-Het al.
Acute human immunodeficiency virus syndrome presenting with hemophagocytic lymphohistiocytosis. Yonsei Med J2008;
49: 325–328.
22.
SunHYet al.
Hemophagocytic lymphohistiocytosis: an unusual initial presentation of acute HIV infection. J Acquir Immune Defic Syndr2004;
37: 1539–1540.
23.
OkabeTet al.
What intensivists need to know about hemophagocytic syndrome: an underrecognized cause of death in adult intensive care units. J Intensive Care Med2012;
27: 58–64.
24.
TothovaZBerlinerN.Hemophagocytic syndrome and critical illness: new insights into diagnosis and management. J Intensive Care Med2015;
30: 401–412.
25.
LiFet al.
Clinical characteristics and prognostic factors of adult hemophagocytic syndrome patients: a retrospective study of increasing awareness of a disease from a single-center in China. Orphanet J Rare Dis2015;
10: 20.
26.
ShawTYSchivoM.Weathering a cytokine storm: a case of EBV-induced hemophagocytic lymphohistiocytosis. J Investig Med High Impact Case Rep2016;
4: 2.
27.
LimSHet al.
Clinical significance of bone marrow hemophagocytosis in adult patients with malignancy and non-malignancy-induced hemophagocytic lymphohistiocytosis. Ann Hematol2016;
95: 325–335.
28.
FerreiraDGet al.
Hemophagocytic lymphohistiocytosis: a case series of Brazilian Institution. Revista Brasileira De Hematologia e Hemoterapia2014;
36: 437–441.
29.
KoduriPRet al.
Reactive hemophagocytic syndrome: a new presentation of disseminated histoplasmosis with AIDS. Clin Infect Dis1995;
21: 1463–1465.
30.
SanchezAet al.
Histoplasmosis-associated hemophagocytic syndrome: a case report. AIDS Read2007;
17: 466–469.