Hemophagocytic Lymphohistiocytosis and Progressive Disseminated Histoplasmosis

Ferguson-Paul, Kenice; Mangum, David Spencer; Porter, Ashley; Leventaki, Vasiliki; Campbell, Patrick; Wolf, Joshua

doi:10.3201/eid2206.151682

Cited by 13 publications

(8 citation statements)

References 8 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The term familial hemophagocytic reticulosis was then changed to primary or familial HL and subsequently, nongenetic causes were recognized (i.e., secondary HLH) [1,4]. While primary HLH consists of different monogenic alterations that derive from impaired cytotoxicity of natural killer cells (NK) and CD8+ cytotoxic T lymphocytes (CTL), secondary HLH presents as an acquired complication in several contexts such as infections, malignant neoplasms, autoimmune diseases, post-transplantation, or is iatrogenically induced (Table 1) [8,9,10,11,12,13,14,15]. Regardless of the etiology, HLH is characterized by an uncontrolled activation of NK/CTL that provokes the release of large amounts of proinflammatory cytokines such as IFN-γ, TNF-α, GM-CSF, M-CSF, and IL-2, resulting in hyperstimulation and systemic infiltration by macrophages which, in turn, phagocytose blood cells, mostly red blood cell precursors, and secrete other cytokines responsible for myelosuppression, endothelial damage with coagulopathy, tissue injury, and NK/CTL incessant activation (IL-1, IL-6, and TNF-α) (Fig.…”

Section: Discussionmentioning

confidence: 99%

Hemophagocytic Lymphohistiocytosis in Adults with Intraocular Involvement: Clinicopathologic Features of 3 Cases

Vizcaíno¹,

Eberhart²,

Rodríguez³

2017

Ocul Oncol Pathol

View full text Add to dashboard Cite

Background/Aims: Hemophagocytic lymphohistiocytosis (HLH) is an infrequent inflammatory multisystemic syndrome. Only rare cases with ophthalmic involvement describing their pathologic features have been previously reported. Methods: We report 3 cases of adult-onset HLH with bilateral ocular involvement and describe their clinicopathologic features. Results: Three adult males - 2 with a history of viral infection - developed persistent fever, fatigue, bone marrow abnormalities, and irreversible multiorgan failure. Visual impairment was also documented in 2 cases. Complete autopsies were performed. Ophthalmic pathology demonstrated a bilateral histiocytic infiltrate with scant lymphocytes affecting the uvea. Focal extension to the retina, optic nerve, and trabecular meshwork were also identified, as well as hemophagocytosis in 1 case. Macrophages showed strong immunoreactivity for CD163 antibody and lacked BRAF p.V600E mutant protein. Conclusion: HLH is an unusual disorder associated with several systemic conditions. Histologic features in the eye are poorly documented, with prior reports restricted to children. Our 3 adult cases are reported using updated criteria and, despite the difference in age, show changes similar to those observed in the pediatric population.

show abstract

Section: Discussionmentioning

confidence: 99%

Hemophagocytic Lymphohistiocytosis in Adults with Intraocular Involvement: Clinicopathologic Features of 3 Cases

Vizcaíno¹,

Eberhart²,

Rodríguez³

2017

Ocul Oncol Pathol

View full text Add to dashboard Cite

show abstract

“…Hemophagocytic syndrome (HPS) or hemophagocytic lymphohistiocytosis is characterized in humans by immune dysregulation that leads to excessive production of inflammatory cytokines, excessive activation of macrophages, and infiltration of well‐differentiated lymphocytes and hemophagocytic macrophages into multiple organs . Acquired human HPS is associated with a variety of conditions, including histoplasmosis . As such, it is possible that acquired HPS could be contributing to some of the cytopenias identified in these cats.…”

Section: Discussionmentioning

confidence: 99%

“…Here, we report a case series of cats in which a Histoplasma ‐like fungal infection was associated with hemophagocytosis in various organs. This occurs occasionally in humans, where histoplasmosis induces a secondary hemophagocytic lymphohistiocytosis . However, to the best of the authors' knowledge, similar feline cases have not been previously published.Cases were identified by retrospective electronic record searches at the clinical pathology laboratories of three different Colleges of Veterinary Medicine: the University of Tennessee, Oklahoma State University, and the University of Missouri.…”

Section: Case Presentationmentioning

confidence: 94%

Hemophagocytosis and Histoplasma‐like fungal infection in 32 cats

Schaefer

Rizzi

Royal

2019

Veterinary Clinical Pathol

View full text Add to dashboard Cite

Histoplasmosis is one of the most common systemic fungal diseases in cats from the United States. It commonly causes respiratory or disseminated disease and is often associated with one or more cytopenias. Here, we describe 32 cats in which a Histoplasma‐like fungal infection was associated with concurrent hemophagia in at least one sample site, commonly spleen, bone marrow, liver, and/or lymph node. The degree of hemophagia was characterized as moderate or marked in the majority of cases, and in all cases, there was a predominance of phagocytized mature erythrocytes. A few cases also had macrophages with phagocytized erythroid precursors, platelets, and/or neutrophils. Complete blood count results were available for 25 cats, and cytopenias were common (20/25), including solitary anemia (10), anemia and thrombocytopenia (5), solitary neutropenia (2), pancytopenia (2), and anemia and neutropenia (1). Bone marrow samples were only available in a small subset of cases, preventing the further assessment of the causes of the cytopenias. Hemophagocytosis has been previously reported in cats with neoplastic diseases and a cat with calicivirus infection, and likely occurs with other conditions as well, such as hemorrhage or hemolysis. Results of this report suggest that systemic fungal disease is an additional differential to consider when there is hemophagia in a feline cytology sample.

show abstract

“…In addition, clinical suspicion for histoplasmosis can be delayed, because many symptoms of disseminated fungal infection and HLH overlap, including fever, hepatosplenomegaly, pancytopenia, and coagulopathy. 3 Our patient was initially treated according to HLH-2004 guidelines with etoposide and high-dose steroids. However, once disseminated histoplasmosis was identified, these immunosuppressive and chemotherapeutic agents were discontinued and the patient was started on antifungal treatment, to which she responded well.…”

Section: Discussionmentioning

confidence: 99%

“…1,2 A rare but serious trigger of HLH is disseminated histoplasmosis, which can be as difficult to diagnose as HLH itself. 3 We present a case of a woman with a history of long-term immunosuppression who developed HLH secondary to disseminated histoplasmosis.…”

mentioning

confidence: 99%

Disseminated infection to immune activation

Yeager

Krenzer

2018

Baylor University Medical Center Proceedings

View full text Add to dashboard Cite

A 61-year-old woman with rheumatoid arthritis on chronic immunosuppression presented with fever. Laboratory studies demonstrated multiorgan failure with pancytopenia, elevation in liver function studies, and an elevated ferritin level, with a diagnosis of hemophagocytic lymphohistiocytosis confirmed on bone marrow biopsy. After initial treatment with chemotherapy, fungal blood cultures grew , prompting initiation of antifungal therapy, which resulted in successful treatment of-associated hemophagocytic lymphohistiocytosis.

show abstract

Hemophagocytic Lymphohistiocytosis and Progressive Disseminated Histoplasmosis

Cited by 13 publications

References 8 publications

Hemophagocytic Lymphohistiocytosis in Adults with Intraocular Involvement: Clinicopathologic Features of 3 Cases

Hemophagocytic Lymphohistiocytosis in Adults with Intraocular Involvement: Clinicopathologic Features of 3 Cases

Hemophagocytosis and Histoplasma‐like fungal infection in 32 cats

Disseminated infection to immune activation

Contact Info

Product

Resources

About