Isolated Bone Marrow Mastocytosis may be Associated with Reduced Overall Survival

Short Communication

Ann Hematol Oncol. 2021; 8(12): 1380.

Isolated Bone Marrow Mastocytosis may be Associated with Reduced Overall Survival

Severino-Freire M1, Damaj GL2, Jendoubi F1, Negretto M1, Laurent C3, De Mas V4, Evrard S3, Apoil PA5, Mailhol C6, Degboe Y7, Dubreuil P8, Hermine O9, Paul C1 and Livideanu CB1*

1Mastocytosis Expert Center (CEREMAST) Toulouse, Department of Dermatology, Toulouse University Hospital, Paul Sabatier University, France

2Departement of Haematology, Caen University Hospital, France

3Department of Anatomy and Pathological Cytology, Toulouse University Hospital, Paul Sabatier University, CRCT INSERM U1037, CEREMAST Toulouse, Toulouse, France

4Department of Haematology, CEREMAST Toulouse, Toulouse, France

5Immunology Clinical Laboratory, CEREMAST Toulouse, Toulouse, France

6Department of Pneumo-Allergology, CEREMAST Toulouse, Toulouse, France

7Department of Rheumatology, CEREMAST Toulouse, Toulouse, France

8CRCM, (CEREMAST, Marseille, Equipe Labellisée Ligue Contre le Cancer), Inserm, Institut Paoli-Calmettes, Aix-Marseille University, CNRS, Marseille, France

9Haematology, CEREMAST Necker, AP-HP, Paris, France

*Corresponding author: Cristina Bulai Livideanu, CEREMAST Toulouse, Department of Dermatology, Toulouse University Hospital, Paul Sabatier University, France

Received: October 01, 2021; Accepted: October 29, 2021; Published: November 05, 2021

Short Communication

Mastocytosis is characterized by accumulation/proliferation of abnormal Mast Cells (MCs) in tissues [1]. Depending on organ involvement, two main forms of mastocytosis are identified Isolated Cutaneous Mastocytosis (ICM), when the skin is the only tissue affected [2,3] and Systemic Mastocytosis (SM), characterized by MC infiltrates in internal organs, mostly the Bone Marrow (BM). SM can be or not associated with concomitant skin involvement. The most frequent form of SM is indolent SM [4].

Isolated Bone Marrow Mastocytosis (IBMM) constitutes a variant of indolent SM described in the 2008 WHO classification for mastocytosis [5]. There are limited information on the course and prognosis of IBMM in the literature. In the literature, it exist for patients with non-advanced and advanced mastocytosis a score who can be used to predict survival outcomes even if the predictive value of the International Prognostic Scoring system for Mastocytosis (IPSM) needs to be confirmed in forthcoming trials [6].

IBMM represents a diagnostic challenge for clinicians as clinical manifestations such as anaphylaxis; osteoporosis and digestive symptoms are not specific.

The main objective of this study was to analyze the overall survival of patients with IBMM in adults with SM. The secondary objectives were to assess the frequency of IBMM in adults with SM, compare the clinical and laboratory characteristics of patients IBBM to those of patients with SM associated cutaneous mastocytosis (SMcm) and evaluate the IPSM prognostic score in our cohort.

Consecutive adult patients with a diagnosis of SM evaluated between January 2009 and January 2020 were included. All patients were prospectively included in the study supported by «French Association for the Initiatives of Research on Mastocyte and Mastocytosis» and entitled “Physiopathological and clinical study of mastocytosis in adult patients”. The study was approved by the Institutional Review Board of Necker Enfants-Malades Hospital on 8th November 2000. The study was carried out in accordance with the Declaration of Helsinki. Written informed consent was obtained from all patients. The diagnosis of SM was made according to the diagnostic criteria defined by the World Health Organization (WHO) [4,5,7,8]. The diagnosis of IBMM was made by one of the board dermatologists with expertise in mastocytosis (MSF, FJ, CP, and/or CBL) after full body examination ruling out visible signs of mastocytosis (Figure 1 and Table 1).

Citation: Severino-Freire M, Damaj GL, Jendoubi F, Negretto M, Laurent C, De Mas V, et al. Isolated Bone Marrow Mastocytosis may be Associated with Reduced Overall Survival. Ann Hematol Oncol. 2021; 8(12): 1380.