CAR-T-cell-related hemophagocytic lymphohistiocytosis ...
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Description

CAR-T-cell-related hemophagocytic lymphohistiocytosis (HLH)/ MAS:

 • Profound systemic inflammatory state characterized by cytotoxic T cell hyperactivation (IFNγ) → macrophages (IL-6), lymphohistiocytic tissue infiltration, and multiorgan failure; develops in ~1% of patients treated with CAR‐T.

 • Definition (proposed): Patient with peak ferritin >10,000 during CRS phase (typically <D5), and two of the following: grade ≥3 organ toxicities involving the liver, kidney, lung; or hemophagocytosis in bone marrow or other organs.

 • Signs/symptoms: Fever, cytopenias (2/3 lines); signs and symptoms related to multiorgan dysfunction

 • Diagnosis: laboratory findings resemble HLH; CBC diff (cytopenia), ferritin (>10,000), sIL2R, LDH, fibrinogen, triglycerides, AST/ALT, Bili, Cr; BMBx rarely critical (low sensitivity, low specificity).

 • Therapy: High mortality, do not delay diagnosis, escalate therapy aggressively (interdisciplinary approach)

    - Manage with anti-IL-6 (tocilizumab) and high-dose glucocorticoids; commonly necessitates additional therapy

    - If failure to improve in 48h, consider etoposide as in HLH-94 treatment protocol; consider intrathecal cytarabine



#CARTcell #hemophagocytic #lymphohistiocytosis #HLHMAS #diagnosis #management #oncology
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Account created for the MGH Internal Medicine Housestaff Manual "White Book" - https://stk10.github.io/MGH-Docs/WhiteBook-2019-2020.pdf
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