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Table 8. Hematologic Toxicities
Grading Management
All Grades • The first step in the management of TTP is a
high index of suspicion for the diagnosis and
timely recognition. Hematolog y consult should
immediately be called, as delay in identification is
associated with increased mortality/morbidity.
• Initially, the patient should be stabilized, and any
critical organ dysfunction stabilized.
G1: Evidence of RBC
destruction (schistocytosis)
without anemia,
renal insufficiency, or
thrombocytopenia clinically
• Hold ICPi and discuss resumption with patient
only after taking into account the risks and benefits,
noting that there is currently no data to recommend
restarting ICPi therapy.
• Administer 0.5–1 mg/kg/day prednisone.
G2: Evidence of RBC
destruction (schistocytosis)
without clinical consequence
with G2 anemia and
thrombocytopenia
G3: Laboratory findings with
clinical consequences (G3
thrombocytopenia, anemia,
renal insufficiency >2)
• Hold ICPi and discuss resumption with patient
only after taking into account the risks and benefits,
noting that there is currently no data to recommend
restarting ICPi therapy.
• In conjunction with hematolog y, initiate therapeutic
plasma exchange (PEX) according to existing
guidelines with further PEX dependent on clinical
progress.
• Administer methylprednisolone 1g intravenously
daily for 3 days, with the first dose typically
administered immediately after the first PEX. For
patient who has an initial platelet count response,
discontinue PEX.
• May offer rituximab
• Consider caplacizumab if ADAMTS13 activity level
is <10 IU/dL or <10% of normal, with an inhibitor
or elevated anti-ADAMTS13 IgG.
• If no exacerbation within 3-5 days after stopping
PEX, taper steroids over 2-3 weeks, complete
course of rituximab (if receiving ) and discontinue
caplacizumab (if receiving ).
G4: Life-threatening
consequences, (e.g., CNS
hemorrhage or thrombosis/
embolism or renal failure)
(cont'd)
