Entry - 246470 - LEUKEMIA, ACUTE MYELOCYTIC, WITH POLYPOSIS COLI AND COLON CANCER - OMIM
 
246470

LEUKEMIA, ACUTE MYELOCYTIC, WITH POLYPOSIS COLI AND COLON CANCER


Clinical Synopsis
 

GI
- Multiple adenomatous colon polyps
- Rectal adenocarcinoma
Heme
- Acute myeloblastic leukemia
GU
- Papillary bladder tumor
Inheritance
- Autosomal recessive

TEXT

In an Italian-American family with first-cousin parents, Greenberg et al. (1981) described 2 brothers, in a sibship of 5, who had this combination. In 1, colectomy with ileoproctostomy was performed for multiple adenomatous polyps of the colon at age 35. Because of multiple polyps and adenocarcinoma in situ in the residual rectum, abdominoperineal resection and ileostomy were done at age 40. Leukemia of acute myeloblastic type was discovered at age 45 and the patient died after a 2-month course. The second brother had fulguration of 2 papillary tumors of the bladder at age 25. In the next 3 years, he had multiple colonic polypectomies and abdominoperineal resection for Duke's stage C-1 carcinoma of the rectum. At age 34 he was discovered to have acute myeloblastic leukemia and died after a very short course.


REFERENCES

  1. Greenberg, M. S., Anderson, K. C., Marchetto, D. J., Li, F. P. Acute myelocytic leukemia in two brothers with polyposis coli and carcinoma of the colon. Ann. Intern. Med. 95: 702-703, 1981. [PubMed: 6946722, related citations] [Full Text]


Creation Date:
Victor A. McKusick : 6/3/1986
mimadm : 2/19/1994
supermim : 3/16/1992
supermim : 3/20/1990
ddp : 10/26/1989
marie : 3/25/1988
reenie : 6/3/1986

246470

LEUKEMIA, ACUTE MYELOCYTIC, WITH POLYPOSIS COLI AND COLON CANCER



TEXT

In an Italian-American family with first-cousin parents, Greenberg et al. (1981) described 2 brothers, in a sibship of 5, who had this combination. In 1, colectomy with ileoproctostomy was performed for multiple adenomatous polyps of the colon at age 35. Because of multiple polyps and adenocarcinoma in situ in the residual rectum, abdominoperineal resection and ileostomy were done at age 40. Leukemia of acute myeloblastic type was discovered at age 45 and the patient died after a 2-month course. The second brother had fulguration of 2 papillary tumors of the bladder at age 25. In the next 3 years, he had multiple colonic polypectomies and abdominoperineal resection for Duke's stage C-1 carcinoma of the rectum. At age 34 he was discovered to have acute myeloblastic leukemia and died after a very short course.


REFERENCES

  1. Greenberg, M. S., Anderson, K. C., Marchetto, D. J., Li, F. P. Acute myelocytic leukemia in two brothers with polyposis coli and carcinoma of the colon. Ann. Intern. Med. 95: 702-703, 1981. [PubMed: 6946722] [Full Text: https://doi.org/10.7326/0003-4819-95-6-702]


Creation Date:
Victor A. McKusick : 6/3/1986

Edit History:
mimadm : 2/19/1994
supermim : 3/16/1992
supermim : 3/20/1990
ddp : 10/26/1989
marie : 3/25/1988
reenie : 6/3/1986