Ruxolitinib as first‐line treatment in secondary hemophagocytic lymphohistiocytosis: A single patient experience

Slostad, Jessica; Hoversten, Patrick; Haddox, Candace L.; Cisak, Kamila I.; Paludo, Jonas; Tefferi, Ayalew

doi:10.1002/ajh.24971

Cited by 52 publications

(42 citation statements)

References 6 publications

(13 reference statements)

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“…Instead, its mechanism of action is based on its nonspecific ability to suppress inflammatory cytokines. Consistent with this impression, the therapeutic value of the drug has also been reported in other inflammation‐related disorders, including graft vs. host disease and secondary hemophagocytic lymphohistiocytosis …”

Section: Risk‐adapted Therapysupporting

confidence: 59%

Primary myelofibrosis: 2019 update on diagnosis, risk‐stratification and management

2018

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Observation alone is advised for MIPSS70+ version 2.0 "low" and "very low" risk disease (estimated 10-year survival 56%-92%); allogeneic stem cell transplant is the preferred treatment of choice for "very high" and "high" risk disease (estimated 10-year survival 0-13%); treatment-requiring patients with intermediate-risk disease (estimated 10-year survival 30%) are best served by participating in clinical trials. All other treatment approaches, including the use of JAK2 inhibitors, are mostly palliative and should not be used in the absence of clear treatment indications. Conventional treatment for anemia includes androgens, prednisone, thalidomide and danazol, for symptomatic splenomegaly hydroxyurea and ruxolitinib and for constitutional symptoms ruxolitinib. Splenectomy is considered for drug-refractory splenomegaly and involved field radiotherapy for nonhepatosplenic EMH and extremity bone pain.

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Section: Risk‐adapted Therapysupporting

confidence: 59%

Primary myelofibrosis: 2019 update on diagnosis, risk‐stratification and management

2018

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“…10,11 Anecdotal experiences of successful use of ruxolitinib to control refractory primary HLH or secondary HLH are also reported in humans. [12][13][14][15][16][17][18][19] JAK1/JAK2 inhibitors are also increasingly used in inflammatory diseases.…”

Section: Introductionmentioning

confidence: 99%

Efficacy of ruxolitinib in subcutaneous panniculitis-like T-cell lymphoma and hemophagocytic lymphohistiocytosis

et al. 2020

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“…Our group first reported first-line use of ruxolitinib in a patient with secondary HLH from disseminated histoplasmosis. 6 The patient was immunosuppressed from methotrexate and hydroxychloroquine use for rheumatoid arthritis. The patient's clinical parameters improved dramatically with a combination of antifungals and ruxolitinib.…”

Section: Ruxolitinib As First-line Treatment In Secondary Hemophagocymentioning

confidence: 99%

Ruxolitinib as first‐line treatment in secondary hemophagocytic lymphohistiocytosis: A second experience

et al. 2018

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Ruxolitinib as first‐line treatment in secondary hemophagocytic lymphohistiocytosis: A single patient experience

Cited by 52 publications

References 6 publications

Primary myelofibrosis: 2019 update on diagnosis, risk‐stratification and management

Primary myelofibrosis: 2019 update on diagnosis, risk‐stratification and management

Efficacy of ruxolitinib in subcutaneous panniculitis-like T-cell lymphoma and hemophagocytic lymphohistiocytosis

Ruxolitinib as first‐line treatment in secondary hemophagocytic lymphohistiocytosis: A second experience

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