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Fahrenheit globulinemia (Waldenström's macroglobulinemia, WM), a rare non-Hodgkin's lymphoma, lymphocyte lymphoma.
A Swedish doctor, Jan G. Waldenström (1906-1996), found that patients showed bleeding from the mouth and nose, anemia, decreased levels of fibrinogen in the blood, swollen lymph nodes, large proliferation of plasma cells in the bone marrow, and blood viscosity due to a large increase in globulin.
(the blood is sticky and difficult to reflow, the fingers become white and cannot be restored for a long time) Bruton tyrosine kinase (BTK) inhibitors exhibit therapeutic activity in Fahrenheit globulinemia.
Zanubrutinib, an selective BTK inhibitor developed by Baiji Shenzhou, China, was approved by the FDA in November 2019 to treat patients with previously treated heterocellular lymphoma (MCL).
Troutman and others conducted a Phase 1/2 clinical trial to assess the efficacy of Zebutinib for primary treatment (TN) or relapse/recurring (R/R) WM.
two Zebtini oral solutions: 160 mg 2/day (n=50) or 320 mg 1/day (n=23).
end points include overall remission rate (ORR) and very good partial/complete remission (VGPR/CR).
September 2014-March 2018, a total of 77 patients (24 TN and 53 R/R) were treated.
36 months and 23.5 months after the mid-level follow-up of R/R patients and TN patients, 72.7% of patients were still receiving treatment.
adverse reactions (13 per cent, 1 treatment-related), disease progression (10.4 per cent) and other (3.9 per cent) causes of termination of treatment.
ORR and VGPR/CR rates were 95.9% and 45.2%, respectively, and VGPR/CR rates increased over time: 20.5% at 6 months, 32.9% at 12 months and 43.8% at 24 months.
the three-year progress-free survival rate is 80.5 per cent and the overall survival rate is 84.8 per cent.
adverse reactions were caused by bruising (32.5 per cent, all level 1), a reduction in neutral granulocytes (18.2 per cent), hemorrhage (3.9 per cent), atrial fibrillation/atrial paralycergic (5.2 per cent) and stage 3 diarrhea (2.6 per cent).
: Some WM patients can obtain deeper lasting relief by using Zebutini monotherapy for a long time.
long-term therapy for these patients is acceptable.
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