Rituximab in steroid refractory autoimmune hemolytic anemia

Information for all healthcare professionals administering this drug: Administer this drug only as an IV infusion. Do not administer as an IV push or bolus. Premedicate before each infusion with acetaminophen and an antihistamine. Pneumocystis jiroveci pneumonia (PCP) is recommended for patients with GPA and MPA during treatment and for at least 6 months following treatment as appropriate.

-FIRST INFUSION: Initiate infusion at a rate of 50 mg/hr; in the absence of infusion toxicity, increase infusion rate by 50 mg/hr increments every 30 minutes, to a maximum of 400 mg/hr
-SUBSEQUENT INFUSIONS: Initiate infusion at a rate of 100 mg/hr; in the absence of infusion toxicity, increase rate by 100 mg/hr increments at 30 minute intervals, to a maximum of 400 mg/hr
-Interrupt the infusion or slow the infusion rate for infusion reactions. Continue the infusion at one-half the previous rate upon improvement of symptoms.

RECOMMENDED DOSE FOR GRANULOMATOSIS WITH POLYANGIITIS (GPA) (WEGENER'S GRANULOMATOSIS) AND MICROSCOPIC POLYANGIITIS (MPA):
-Administer rituximab as a 375 mg/m2 intravenous infusion once weekly for 4 weeks
-Glucocorticoids administered as methylprednisolone 1000 mg IV per day for 1 to 3 days followed by oral prednisone 1 mg/kg/day (not to exceed 80 mg/day and tapered per clinical need) are recommended to treat severe vasculitis symptoms. This regimen should begin within 14 days prior to or with the initiation of rituximab and may continue during and after the 4 week course of rituximab treatment.
-Safety and efficacy of treatment with subsequent courses of this drug have not been established

Use: This drug, in combination with glucocorticoids, is indicated for the treatment of adult patients with Granulomatosis with Polyangiitis (GPA) (Wegener's Granulomatosis) and Microscopic Polyangiitis (MPA)

In a phase II clinical trial, Martinez et al (2009) examined the safety and effectiveness of alemtuzumab in treating steroid-refractory acute GVHD (aGVHD) grade II or higher after stem cell transplantation.  A total of 10 adult patients (6 with aGVHD grade III and 4 with aGVHD grade IV) were included in the study.  Nine patients had gastrointestinal tract involvement, 7 had skin involvement, and 5 had liver involvement.  Five patients responded to treatment, 2 with CR and 3 with partial response.  Eight infectious events (4 of grade 3 to 4) and 7 CMV re-activations were observed.  Six patients had grade 3 to 4 cytopenia.  All 10 patients died (7 resulting from aGVHD progression, 2 from severe infection, and 1 from to leukemia relapse), at a median of 40 days (range of 4 to 88 days) after alemtuzumab treatment.  Overall, these findings suggested that steroid-refractory aGVHD may be improved by treatment with alemtuzumab, but that this treatment does not overcome the dismal prognosis of patients with severe aGVHD, demonstrating the need for alternative therapies to treat this complication.

The treatment and prognosis of NSIP will be reviewed here. The clinical manifestations, evaluation, and diagnosis of NSIP and the diagnosis and management of the other IIPs are discussed separately. (See "Causes, clinical manifestations, evaluation, and diagnosis of nonspecific interstitial pneumonia" and "Idiopathic interstitial pneumonias: Clinical manifestations and pathology" and "Clinical manifestations and diagnosis of idiopathic pulmonary fibrosis" and "Treatment of idiopathic pulmonary fibrosis" and "Respiratory bronchiolitis-associated interstitial lung disease" and "Cryptogenic organizing pneumonia" and "Acute interstitial pneumonia (Hamman-Rich syndrome)" .)

Rituximab in steroid refractory autoimmune hemolytic anemia

rituximab in steroid refractory autoimmune hemolytic anemia

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