PB2081 HIGH INCIDENCE OF MALIGNANCY IN THE FAMILIES OF PATIENTS WITH MYELODYSPLASTIC SYNDROMES
Authors: Hilal A. , Dillion R. , Shepherd N. , Roberts A. , Raj K. 
Published: June 2019

Background:

Myelodysplastic syndromes (MDS) are a group of clonal disorders characterized by bone marrow failure, a propensity for developing leukaemia & infections. As MDS may develop in patients with an inherited tendency to cancer e.g.telomeropathy, we undertook a survey of patients in our clinic to understand whether patients with MDS had an increased family history of malignancy

Aims:

To assess the incidence of malignancy in the family of patients with MDS

Methods

We performed a retrospective review of 80 patients with MDS diagnosed between 2010 ‐2018, noting comorbid malignancy in the individual & history of malignancy in the extended family with the relationship & the type of malignancy.

Results:

Patients were median of 73 years (range 26‐ 92 years). 60.7% were Male. Patients were mainly Caucasian (77%), 15% were Afro Caribbean & 7% Asian. Patients were IPSS: 54% LOW, 30% INT‐1, 11% INT‐2, 5% HIGH and rIPSS: 38% V LOW, 43% LOW, 15% INT, 1% HIGH, 3% V HIGH. Prior malignancy was noted in 24%: Prostate cancer (26%), colo‐rectal & basal cell carcinoma 16% each. Among the 24% patients with comorbid cancers, 16% had a history of more than 1 malignancy along with MDS. Prostate cancer was seen in each of them. Namely prostate & pancreatic cancer, another prostate & large B cell lymphoma of the tongue and another with prostate, colorectal & basal cell carcinoma. In 40% of the cases a detailed family history was not available. 5% were not in contact with their families. 14% had no family history of cancer but had cardiovascular, metabolic disease, asthma or stroke. 40% had a very strong family history of malignancies. Half of these (20%) had a history of hematologic malignancy in both 1st degree & 2nd degree relatives with leukemia 13%, lymphoma 4%, MDS 2% and unclassified 1%. The remaining 20% had a family history of solid organ malignancy with 16.25% having an overlap of more than one cancer. These included; breast cancer 3.98%, bowel and lung cancer 3.59% each, brain and gastric cancer 1.57% each, unknown 1.71%, liver and cervical cancer 0.80% each, melanoma, tonsils, pancreatic, esophageal, throat and ovarian tumors 0.40% each. The median number of relatives affected by cancer was 2 in each patient. Younger patients had increased numbers of relatives with malignancy. They included a 26 years old had a sister with AML, cytopenia in parents and lung cancer in second degree relatives. Patient aged 29 had two second degree relatives who died of bowel cancer, patients aged 28 had parent with esophageal carcinoma and 3 second degree relatives who died of malignancies of breast, stomach and lungs. A patient presented at the age of 29 had one relative with breast malignancy.

Summary/Conclusion:

We show that upto 40% of patients with MDS including those presenting at an older age have a strong family history of malignancy. (In half, this is another haematological malignancy but in half these are solid organ tumours most commonly breast, bowel and lung cancer) In recent times next generation sequencing has yielded information on somatic mutation in MDS however these do delineate genetic predisposition to malignancy. Guidance on screening patients who have a genetic predisposition is available and we suggest whole exome sequencing of families may yield further information.