Successful Modified Therapy in a Patient with Probable Infection-Associated Hemophagocytic Lymphohistiocytosis
Hemophagocytic lymphohistiocytosis (HLH) is a rare, hyperinflammatory syndrome characterized by clinical signs and symptoms of extreme inflammation. In adults, HLH is typically a complication of infections, autoimmune diseases, and malignancies. While the disease is often fatal, classic management o...
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| Format: | Article |
| Language: | English |
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Wiley
2019-01-01
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| Series: | Case Reports in Oncological Medicine |
| Online Access: | http://dx.doi.org/10.1155/2019/9781065 |
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| author | Carl L. Kay Matthew J. Rendo Paul Gonzales Sead G. Beganovic Magdalena Czader |
| author_facet | Carl L. Kay Matthew J. Rendo Paul Gonzales Sead G. Beganovic Magdalena Czader |
| author_sort | Carl L. Kay |
| collection | DOAJ |
| description | Hemophagocytic lymphohistiocytosis (HLH) is a rare, hyperinflammatory syndrome characterized by clinical signs and symptoms of extreme inflammation. In adults, HLH is typically a complication of infections, autoimmune diseases, and malignancies. While the disease is often fatal, classic management of HLH revolves around early diagnosis and initiation of protocolized therapy. We present a case of a previously healthy 56-year-old female who developed distributive shock requiring intubation, vasopressors, and continuous venovenous hemofiltration. In the setting of multiple infectious syndromes, severe cytopenias, and rising direct hyperbilirubinemia, her diagnosis of HLH was confirmed. Therapy was initiated with dexamethasone and two doses of reduced-intensity etoposide based on the patient’s clinical course. Over the next few weeks, she continued to improve on dexamethasone monotherapy and has maintained remission up to the present with complete resolution of her cytopenias and return of baseline renal function. Our case highlights the variability in the management of probable infection-associated HLH (IHLH) with a good patient outcome. We demonstrate the potential to treat IHLH with partial protocols and minimal chemotherapeutics. |
| format | Article |
| id | doaj-art-bc3dfe197c5f47e5801f11db591aa647 |
| institution | Kabale University |
| issn | 2090-6706 2090-6714 |
| language | English |
| publishDate | 2019-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Oncological Medicine |
| spelling | doaj-art-bc3dfe197c5f47e5801f11db591aa6472025-02-03T06:14:09ZengWileyCase Reports in Oncological Medicine2090-67062090-67142019-01-01201910.1155/2019/97810659781065Successful Modified Therapy in a Patient with Probable Infection-Associated Hemophagocytic LymphohistiocytosisCarl L. Kay0Matthew J. Rendo1Paul Gonzales2Sead G. Beganovic3Magdalena Czader4Brooke Army Medical Center, Ft. Sam Houston, TX, USABrooke Army Medical Center, Ft. Sam Houston, TX, USABrooke Army Medical Center, Ft. Sam Houston, TX, USAHematology-Oncology, Indiana University School of Medicine, Indianapolis, IN, USAHematopathology, Indiana University School of Medicine, Indianapolis, IN, USAHemophagocytic lymphohistiocytosis (HLH) is a rare, hyperinflammatory syndrome characterized by clinical signs and symptoms of extreme inflammation. In adults, HLH is typically a complication of infections, autoimmune diseases, and malignancies. While the disease is often fatal, classic management of HLH revolves around early diagnosis and initiation of protocolized therapy. We present a case of a previously healthy 56-year-old female who developed distributive shock requiring intubation, vasopressors, and continuous venovenous hemofiltration. In the setting of multiple infectious syndromes, severe cytopenias, and rising direct hyperbilirubinemia, her diagnosis of HLH was confirmed. Therapy was initiated with dexamethasone and two doses of reduced-intensity etoposide based on the patient’s clinical course. Over the next few weeks, she continued to improve on dexamethasone monotherapy and has maintained remission up to the present with complete resolution of her cytopenias and return of baseline renal function. Our case highlights the variability in the management of probable infection-associated HLH (IHLH) with a good patient outcome. We demonstrate the potential to treat IHLH with partial protocols and minimal chemotherapeutics.http://dx.doi.org/10.1155/2019/9781065 |
| spellingShingle | Carl L. Kay Matthew J. Rendo Paul Gonzales Sead G. Beganovic Magdalena Czader Successful Modified Therapy in a Patient with Probable Infection-Associated Hemophagocytic Lymphohistiocytosis Case Reports in Oncological Medicine |
| title | Successful Modified Therapy in a Patient with Probable Infection-Associated Hemophagocytic Lymphohistiocytosis |
| title_full | Successful Modified Therapy in a Patient with Probable Infection-Associated Hemophagocytic Lymphohistiocytosis |
| title_fullStr | Successful Modified Therapy in a Patient with Probable Infection-Associated Hemophagocytic Lymphohistiocytosis |
| title_full_unstemmed | Successful Modified Therapy in a Patient with Probable Infection-Associated Hemophagocytic Lymphohistiocytosis |
| title_short | Successful Modified Therapy in a Patient with Probable Infection-Associated Hemophagocytic Lymphohistiocytosis |
| title_sort | successful modified therapy in a patient with probable infection associated hemophagocytic lymphohistiocytosis |
| url | http://dx.doi.org/10.1155/2019/9781065 |
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