62
Treatment
Table 8. Hematologic Toxicities
G2: Moderate: 1–5% of
normal factor activity in
blood;
0.01–0.05 IU/ml of whole
blood
• Hold ICPi and discuss resumption with patient only
after taking into account the risks and benefits.
• Administration of factor replacement (choice based
on BU of titer).
• Administer 1 mg/kg/day prednisone ± rituximab
(dose 375 mg/m
2
weekly × 4 weeks) and/or
cyclophosphamide (dose 1–2 mg/kg/day). Choice
of rituximab vs cyclophosphamide is patient
specific and should be done with assistance of
hematolog y consult. Prednisone, rituximab, and
cyclophosphamide should be given for at least 5
weeks.
• Factors should be provided to increase level during
bleeding episodes, with choice of factor based on
presence or absence of inhibitor.
• Transfusion support as required for bleeding.
G3–4: Severe: <1% of
normal factor activity in
blood; <0.01 IU/ml of whole
blood
• Permanently discontinue ICPi.
• Admit patient.
• Administration of factor replacement, choice based
on BU level of inhibitor.
• Bypassing agents may be used (Factor VII FEIBA).
Caution should be taken in elderly patients and those
with CAD.
• Prednisone corticosteroids 1–2 mg/kg/day (oral
or IV depending on symptoms) ± rituximab
(dose 375 mg/m
2
weekly × 4 weeks) and/or
cyclophosphamide (dose 1–2 mg/kg/day).
• Transfusion support as required for bleeding.
• If worsening or no improvement add cyclosporine or
immunosuppression/ immunoadsorption.
Additional considerations:
• Acquired Hemophilia A requires special clinical and laboratory expertise. Consult
and/or transfer to a specialist center is often appropriate. If consultation with or
transfer to a hemophilia center is not immediately possible, then investigation and
treatment should be initiated while a liaison is being established.
(cont'd)
