Blood: The efficacy of BTKi, a self-developed drug, in the long-term treatment of Fahrenheit globulinemia.

Waldenstr?m's macroglobulinemia,WM), a rare non-Hodgkin's lymphoma, a lymphatic plasma cell lymphoma.
was found by a Swedish doctor, Jan G. Waldenstr?m (1906-1996), and the patients showed oral and nasal bleeding, anemia, reduced levels of fibroproteinogens in the blood, swollen lymph nodes, a large proliferation of plasma cells in the bone marrow, and blood viscosity due to a large increase in cytoglobin.
(blood is sticky and difficult to return, fingers will turn white and cannot be recovered for a long time) Bruton tyrosine kinase (BTK) inhibitors show therapeutic activity in Fahrenheit globulinemia.
zanubrutinib, a selective BTK inhibitor developed by Baiji Shenzhou, China, was approved by the FDA in November 2019 to treat patients with previously treated set of cellular lymphoma (MCL).
Trotman and others conducted a Phase 1/2 clinical trial to assess the efficacy of Zebuteni for primary treatment (TN) or recurrent/incurable (R/R) WM.
are two types of Zebutini 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.
patients with R/R and TN were followed for 36 months and 23.5 months, respectively, 72.7% of patients were still receiving treatment.
the termination of treatment were adverse reactions (13 per cent, 1 treatment-related), disease progression (10.4 per cent) and others (3.9 per cent).
ORR and VGPR/CR rates were 95.9 per cent and 45.2 per cent, respectively, and the VGPR/CR rates increased over time: 20.5 per cent at 6 months, 32.9 per cent at 12 months and 43.8 per cent at 24 months.
the three-year non-progress 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), reduction of neutral granulocytes (18.2 per cent), haemorrhage (3.9 per cent), atrial fibrillation/atrial attack (5.2 per cent) and level 3 diarrhoea (2.6 per cent).
conclusion: Some WM patients can obtain deeper long-lasting remission by long-term treatment with Zebutinib monotherapy.
long-term therapy for this type of patient is acceptable.
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