CM-06. Mastocytosis in adults: A retrospective cohort study
Vsevolod G. Potapenko1,2, Anna V. Klimovich1, Irena E. Belousova4, Anna M. Maximova4, Ekaterina A. Ukrainchenko3, Alfia S. Nizamutdinova3, Ksenia A. Skoryukova1, Elena V. Lisukova2, Tatiana G. Kulibaba5, Maria V. Barabanshchikova2, Elena V. Morozova2, Nadezhda V. Medvedeva1
1 Municipal Clinical Hospital No. 31, St. Petersburg, Russia
2 RM Gorbacheva Research Institute of Pediatric Oncology, Hematology and Transplantation, Pavlov University, St. Petersburg, Russia
3 Aleksandrovsky Hospital, St. Petersburg, Russia
4 S. M. Kirov Military Medical Academy, St. Petersburg, Russian Federation
5 Saint Petersburg State University, St. Petersburg, Russia
Contact: Dr. Vsevolod G. Potapenko, e-mail: potapenko.vsevolod@mail.ru
Summary
Introduction
Mastocytosis is a clonal disease with mast cells proliferation and storage. Patients with cytopenia, ascites, severe bone or bowel impairment as an aggressive course (AMС) need the anticancer treatment. In non-aggressive cases (NAM), only symptom-reducing treatment is recomended. Our aim was to summarize clinical features and treatment outcome in adult mastocytosis patients.
Patients and methods
A retrospective study was performed by analyzing medical documents and questionnaires of patients who attended at our clinics (n=32), or distantly consulted patients (n=21) since Apr 2008 until Feb 2020.
Results
The study included fifty-three patients: 36 females (70%) and 17 males (30%) living in Russia and neighboring countries. Median follow-up time was 56.5 (3 to 564) months. Median age at diagnosis was 39 y.o. (1-79), the age of study inclusion was 39 (18-79) years. All the patients exhibited skin rash. The diagnostic procedures are listed in Table 1. Main clinical features and complaints of the patients with mastocytosis are listed in Table 2.
Main therapeutic approaches in the NAM patients is listed in Table 3. Only 2 patients (4.4%) were dissatisfied by their life quality.
Fig. 1 and 2 show the dynamic changes of complaints and skin rash. Almost all patients are alive during all follow-up period; only one patient died, due to progression of associated chronic myelomonocytic leukemia. Aggressive mastocytosis was diagnosed in eight patients (15%), three of them developed mast cell leukemia. The cytoreductive treatment was started, as recommended in cases of cytopenia (n=3), or symptomatic osteolysis (n=2), and portal hypertension syndrome (n=3). Overall survival in this group was 62.5% (n=5), median follow-up 80 (12-131) months. Overall survival in patients with NAM and aggressive mastocytosis is presented in Fig. 3.
Conclusion
The presented data confirm the benign prognosis of NAM. Antihistamine drugs are effective to maintain life quality in the majority of patients.
Anticancer treatment can provide significant long lasting improvement in a proportion of patients with aggressive mastocytosis.
Keywords
Mastocytosis, adults, symptoms, treatment, survival.
