School of Medicine and Health Sciences Poster Presentations

Title

Acquired hemophagocytic lymphohistiocytosis: Identifying the trigger in a rare hyperinflammatory disorder

Poster Number

284

Document Type

Poster

Status

Medical Resident

Abstract Category

Immunology/Infectious Diseases

Keywords

hemophagocytic ,lymphohistiocytosis,HLH,Histoplasmosis,interleukin-2

Publication Date

Spring 2018

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare disease entity characterized by abnormal activation of the immune system, resulting in unchecked proinflammatory cytokine release and pathologic amplification. Untreated HLH has high mortality rates. HLH may be classified as a primary (genetic) or secondary (acquired) disorder. A variety of pathological states may act as a trigger for acquired HLH, including infections, malignancies, and autoimmune diseases.

A 47-year old African-American female kidney transplant recipient with systemic lupus erythematosus (SLE) and end-stage renal disease (ESRD), was admitted to our center with fever, chills and nasal congestion, diarrhea and anorexia. The patient had a living kidney donor transplant in 2001 and a deceased donor kidney transplant in 2016. Her immunosuppressive treatment on admission consisted of oral prednisone, mycophenolate mofetil and tacrolimus.

Clinical and laboratory findings were suggestive of sepsis. Empiric broad-spectrum intravenous antibiotics were started and immunosuppressive therapy reduced accordingly. Viral work up was negative for BK virus, hepatitis B virus, hepatitis C virus, Epstein-Barr virus and human immunodeficiency virus however cytomegalovirus testing was positive. Bacterial infection was excluded on serial blood cultures. Antiviral therapy with valganciclovir was initiated but she remained febrile. In the context of therapy-refractory fever and pancytopenia with disproportionately elevated levels of ferritin, lactate dehydrogenase, triglycerides and soluble interleukin-2 receptor (sIL-2r/sCD25) a suspected diagnosis of HLH was established.

CT chest/abdomen/pelvis identified a new cluster of small nodules in the peripheral right upper lobe, small right pleural effusion and multiple old mediastinal lymph nodes, but no hepatosplenomegaly. CT Neck revealed a prevertebral effusion with endplate erosions at C4-C5 level. This was followed up with an MRI Neck that showed end-plate edema without significant edema in the disc space.

Histoplasma capsulatum species was isolated from bone marrow culture. There was no evidence for viral, bacterial, parasitic infection, malignancy or active SLE.

After initiation of liposomal amphotericin therapy fevers resolved and treatment was transitioned to oral itraconazole. Laboratory parameters including ferritin, LDH, triglycerides and all cell lines improved. Given patients clinical response to antifungal therapy, the development of HLH was attributed to disseminated histoplasmosis. The patient was discharged 43 days after the initial admission.

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Acquired hemophagocytic lymphohistiocytosis: Identifying the trigger in a rare hyperinflammatory disorder

Hemophagocytic lymphohistiocytosis (HLH) is a rare disease entity characterized by abnormal activation of the immune system, resulting in unchecked proinflammatory cytokine release and pathologic amplification. Untreated HLH has high mortality rates. HLH may be classified as a primary (genetic) or secondary (acquired) disorder. A variety of pathological states may act as a trigger for acquired HLH, including infections, malignancies, and autoimmune diseases.

A 47-year old African-American female kidney transplant recipient with systemic lupus erythematosus (SLE) and end-stage renal disease (ESRD), was admitted to our center with fever, chills and nasal congestion, diarrhea and anorexia. The patient had a living kidney donor transplant in 2001 and a deceased donor kidney transplant in 2016. Her immunosuppressive treatment on admission consisted of oral prednisone, mycophenolate mofetil and tacrolimus.

Clinical and laboratory findings were suggestive of sepsis. Empiric broad-spectrum intravenous antibiotics were started and immunosuppressive therapy reduced accordingly. Viral work up was negative for BK virus, hepatitis B virus, hepatitis C virus, Epstein-Barr virus and human immunodeficiency virus however cytomegalovirus testing was positive. Bacterial infection was excluded on serial blood cultures. Antiviral therapy with valganciclovir was initiated but she remained febrile. In the context of therapy-refractory fever and pancytopenia with disproportionately elevated levels of ferritin, lactate dehydrogenase, triglycerides and soluble interleukin-2 receptor (sIL-2r/sCD25) a suspected diagnosis of HLH was established.

CT chest/abdomen/pelvis identified a new cluster of small nodules in the peripheral right upper lobe, small right pleural effusion and multiple old mediastinal lymph nodes, but no hepatosplenomegaly. CT Neck revealed a prevertebral effusion with endplate erosions at C4-C5 level. This was followed up with an MRI Neck that showed end-plate edema without significant edema in the disc space.

Histoplasma capsulatum species was isolated from bone marrow culture. There was no evidence for viral, bacterial, parasitic infection, malignancy or active SLE.

After initiation of liposomal amphotericin therapy fevers resolved and treatment was transitioned to oral itraconazole. Laboratory parameters including ferritin, LDH, triglycerides and all cell lines improved. Given patients clinical response to antifungal therapy, the development of HLH was attributed to disseminated histoplasmosis. The patient was discharged 43 days after the initial admission.