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[위암 251 - Mantle cell lymphoma]

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1. Mantle zone은 어디인가? Mantle cell lymphoma의 족보는?


2. Mantle cell lymphoma의 임상적 특징

가장 중요한 특징은 처음 발견시 systemic disease인 경우가 대부분이라는 것입니다. 위장관도 대부분의 경우에 이미 침윤되어 있다고 합니다 (World J Gastroenterol. 2010).

1) Subtype of B cell NHL

2) Typically present with widespread adenopathy and frequently have bone marrow and extranodal involvement (Lymph nodes > 90%, Bone marrow 70-80%, Spleen 60%, Liver 30%, GI tract 20-30%), Peripheral blood (lymphocytosis) 20-30%)

3) GI tract involvement: 과거에는 10-20% 정도로 생각하였으나 최근에는 80-90%로 보고 있음

4) Most common type in GI tract: lymphomatous polyposis

5) Pathologically low-grade, clinically high-grade

6) 3-10% of all lymphoma cases

7) Mean age at presentation: 60 years

8) M:F = 3:1

9) Stage III-IV at presentation (70-80% of cases)

10) Poor prognosis: 5 years OS ≤ 30%

11) Best treatment modality: uncertain

lymphomatous polyposis 형태의 mantle cell lymphoma


3. Mantle cell lymphoma of the GI tract

1) Most common site: ileo-cecal region

2) Any other area may be involved: from the stomach to the rectum

3) Most patients have extensive nodal and bone marrow disease at presentation

4) Symptoms: abdominal pain, diarrhea, hematochezia --> non-specific

다장기 침범은 일반적인 현상입니다. 심지어 육안소견이 정상인 경우라도 조직검사를 하면 십이지장이나 대장 침범이 흔히 발견됩니다.

Duodenum, stomach and colon involvement of MCL

© 2015-4-23. 이준행