Intestinal marginal zone B-cell lymphoma of MALT type: clinical manifestation and outcome of a rare disease.
Intestinal marginal zone B-cell lymphoma of the MALT type (I-MZL) is a relatively uncommon form of lymphoma. Twenty-seven patients with histologically-confirmed I-MZL were analyzed. The patients initially presented with abdominal pain (62.9%), and diarrhea (22.2%). The most common involved site was the ileo-caecal area (40.7%). Musshoff's stage I(E), II(E)1, II(E)2, III(E) and IV were present in 44%, 15%, 11%, 7.4% and 22% respectively. Sixty-three percent were in the low-risk group according to the Follicular Lymphoma International Prognostic Index. Complete response and partial response were achieved in 82% and 4% patients. The estimated 5-year overall survival (OS) and progression-free survival (PFS) rates were 86% and 54%. Stage > or = II(E)2 was determined to be a poor prognostic factor for PFS and OS. I-MZL commonly manifests in an early-stage, low-risk state and tends to respond well to local and systemic treatment with favorable prognosis. I-MZL tends to be an indolent disease - characterized by prolonged survival with frequent relapses, similarly to other site MZLs.
Oh
Sung Yong
SY
Department of Internal Medicine, Dong-A University College of Medicine, Busan, Korea.
Kwon
Hyuk-Chan
HC
Kim
Won Seog
WS
Hwang
In Gyu
IG
Park
Yeon Hee
YH
Kim
Kihyun
K
Ko
Young Hae
YH
Ryoo
Baek-Yeol
BY
Kang
Hye Jin
HJ
Nam
Eunmi
E
Lee
Jae-Hoon
JH
Kim
Jung Han
JH
Kim
Hyo-Jin
HJ
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002430:Cecal Neoplasms / Q000175:diagnosis / Q000401:mortality* / Q000473:pathology / Q000628:therapy; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007078:Ileal Neoplasms / Q000175:diagnosis / Q000401:mortality* / Q000473:pathology / Q000628:therapy; D018442:Lymphoma, B-Cell, Marginal Zone / Q000175:diagnosis / Q000401:mortality* / Q000473:pathology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D035583:Rare Diseases / Q000175:diagnosis / Q000401:mortality* / Q000473:pathology / Q000628:therapy; D012008:Recurrence; D012307:Risk Factors; D015996:Survival Rate