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[Mantle cell lymphoma]

1. Mantle zoneÀº ¾îµðÀΰ¡? Mantle cell lymphomaÀÇ Á·º¸´Â?

2. Mantle cell lymphomaÀÇ ÀÓ»óÀû Ư¡

3. GI involvement of nodal MCL

4. Colon involvement of MCL : lymphomatous polyposis type

5. Colon involvement of MCL : non-lymphomatous polyposis type

6. Stomach involvement of MCL

7. Pathology and genetics

8. Cyclin D negative mantle cell lymphoma

9. GI mantle cell lymphoma, experiences at Samsung Medical Center


1. Mantle zoneÀº ¾îµðÀΰ¡? Mantle cell lymphomaÀÇ Á·º¸´Â?

Small B-Cell Lymphomas - Rebecca L. King MD, Paul J. Kurtin MD, in Hematopathology (Third Edition), 2018

Mantle cell lymphoma cells express pan B-lymphocyte antigens such as CD19, CD20, CD79a, CD79b, and PAX-5. They are positive for IgM or IgM plus IgD and exhibit immunoglobulin light chain restriction. More MCL cases express lambda immunoglobulin light chain than kappa immunoglobulin light chain. Greater than 95% are positive for CD5 but negative for other T-cell antigens, such as CD3, and most either completely lack CD23 expression or show weak CD23 marking in a subset of the tumor cells. They typically lack or have low-intensity expression of CD200 and are most often LEF1-negative, both of which may aid in the distinction from CLL/SLL. They are usually also negative for CD10 and BCL6. Exceptions to this typical phenotype exist. CD5- MCL cases have been described. They can be recognized on the basis of typical morphologic features and expression of cyclin D1 and will have the classic t(11;14)(q13;q32) CCND1/IGH as described below. A very small number of MCLs is positive for the follicle center cell marker BCL6, and MCL is one of the types of B-cell lymphomas that can co-express both CD5 and CD10. As a consequence of the t(11;14)(q13;q32) (see below), the nuclei of the majority of MCLs are positive for cyclin D1, a marker demonstrated best by paraffin section immunohistochemistry. More recently, cyclinD1-negative MCLs have been described, which can pose a diagnostic challenge to the pathologist. Expression of SOX11 by immunohistochemistry is seen in a high proportion (>90%) of MCL and appears useful in recognition of cyclin D1 negative MCL. Interestingly, SOX11-negative cases seem to represent a subtype of MCL, which has a more indolent course, leukemic/non-nodal involvement, and mutated IGHV. SOX11 is not expressed in other small B-cell lymphomas or DLBCL (including CD5+ DLBCL). It can be expressed in some cases of Burkitt lymphoma and lymphoblastic lymphoma, but these entities are not typically in the differential diagnosis of MCL.


2. Mantle cell lymphomaÀÇ ÀÓ»óÀû Ư¡

°¡Àå Áß¿äÇÑ Æ¯Â¡Àº óÀ½ ¹ß°ß½Ã systemic diseaseÀÎ °æ¿ì°¡ ´ëºÎºÐÀ̶ó´Â °ÍÀÔ´Ï´Ù. À§Àå°üµµ ´ëºÎºÐÀÇ °æ¿ì¿¡ ÀÌ¹Ì Ä§À±µÇ¾î ÀÖ´Ù°í ÇÕ´Ï´Ù (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: °ú°Å¿¡´Â 10-20% Á¤µµ·Î »ý°¢ÇÏ¿´À¸³ª ÃÖ±Ù¿¡´Â 80-90%·Î º¸°í ÀÖÀ½

4) Most common type in GI tract: lymphomatous polyposis

ÁÂÃø »çÁøÀ» º¸½Ê½Ã¿À. Áö±Ý±îÁö Á¦°¡ º» Áõ·Ê Áß °¡Àå ÀüÇüÀûÀÎ lymphomatous polyposis ÇüÅÂÀÇ mantle cell lymphomaÀÔ´Ï´Ù.

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 ¡Â 30%

11) Best treatment modality: uncertain


[ÇÑ oncologistÀÇ ¼³¸í]

Mantle cell lymphoma ´Â ±âº»ÀûÀ¸·Î t(11;14)¿¡ ÀÇÇؼ­ cyclin D1 ÀÌ ¾ç¼ºÀÌ°í, ¾ç¼ºÀÌ ¾Æ´Ñ °æ¿ì´Â cyclin D2 or 3°¡ ¾ç¼ºÀÏ °ÍÀ¸·Î »ý°¢ÇÕ´Ï´Ù. ´ë°³ µÎ°¡Áö ŸÀÔÀ¸·Î ³ª´¯´Ï´Ù.

1) Nodal type

ÀÌ °æ¿ì´Â oncogenic transcription factor ÀÎ SOX11 ¾ç¼ºÀÌ°í ÀϹÝÀûÀ¸·Î Àü½Å ´Ù¹ß¼º ¸²ÇÁÀý ºñ´ë¸¦ º¸À̸ç ÀÓ»óÀûÀ¸·Î´Â Follicular lymphoma ¿Í DLBCLÀÇ Áß°£ Á¤µµµÇ´Â °ø°Ý¼ºÀ» º¸ÀÔ´Ï´Ù. µû¶ó¼­ ÀÓ»óÀûÀÎ ¸ð½ÀÀº mantle cellÀº ±×·¸°Ô ºü¸£°Ô Áõ½ÄÇÏ°í °ø°Ý¼ºÀ» óÀ½ºÎÅÍ º¸ÀÌÁö´Â ¾Ê½À´Ï´Ù. °ú°Å mantle cellÀ» low grade·Î ºÐ·ùÇß´ø °Íµµ ÀÌ·± ÀÌÀ¯ÀÌÁö¿ä. ÇÏÁö¸¸ Àç¹ßÀ» ¹Ýº¹Çϸ鼭 °ø°ÝÀûÀ¸·Î º¯ÇØ°¡±â ¶§¹®¿¡ (´Ù¹ß °ñ¼öÁ¾ ó·³) óÀ½ºÎÅÍ Àû±ØÀûÀÎ Ä¡·á¸¦ ÇÕ´Ï´Ù. Áõ»óÀÌ ¸¹ÀÌ ½ÉÇÏÁö ¾Ê¾Æµµ...

2) Leukemic non-nodal type

ÀÌ °æ¿ì´Â memory-like B cell orginÀ¸·Î SOX11 À½¼ºÀÌ°í ÀÌ °æ¿ì´Â ÁÖ·Î CLL ó·³ ¸»ÃÊ Ç÷¾×¿¡ ºñÁ¤»ó¼¼Æ÷°¡ ¸¹½À´Ï´Ù. ±×·±µ¥ ´ë°³ indolent Çؼ­ Ä¡·á¸¦ ÇÏÁö ¾Ê°í °æ°ú¸¦ ¸¹ÀÌ º¾´Ï´Ù. ¹°·Ð ÀÌ °æ¿ìµµ Ä¡·á°¡ ÇÊ¿äÇÑ °æ¿ì¿¡´Â ÇÕ´Ï´Ù. CLLó·³ matnle cel ´ëÇ¥ÀûÀ¸·Î À§Àå°ü multiple polyp À» º¸ÀÌ´Â °æ¿ì°¡ ¸¹Àºµ¥ ´ëºÎºÐ 1)¿¡¼­ ±×·¸°í ÀÌ°Í ¿©ºÎ°¡ ¿¹ÈÄÀÚü¿¡ ¹ÌÄ¡´Â ¿µÇâÀº Å©Áö ¾Ê½À´Ï´Ù.

»ç½Ç stage I/II mantle cell lymphoma´Â ¸Å¿ì Èñ±ÍÇÕ´Ï´Ù. Áõ»óÀÌ ¾ø¾î¼­ Áø´Ü½Ã¿¡ ´ë°³ 4±âÀÔ´Ï´Ù.


3. GI involvement of nodal MCL (Salar A. Am J Surg Pathol 2006;30(10):1274-80)

1) Only 26% of patients presented with GI symptoms at the time of diagnosis.

2) MCL was present histologically in the lower GI tract of 53 of 60 patient (88%) and in the upper GI tract of 28 of 58 patients (43%).

3) Microscopic evidence of MCL was found in 84% of patients with normal visual (macroscopic) findings by lower endoscopy and in 45% of patients with macroscopically normal findings by upper endoscopy.

4) Abnormal mucosa was identified in 38% of cases by upper endoscopy (mainly mild nonspecific gastritis) and in 54% of cases by lower endoscopy (mostly micropolyps).

Duodenum, stomach and colon involvement of MCL

5) Histologically, infiltration by MCL was demonstrated in the stomach in 77% of cases and in the colon in 77% of cases.

6) As a whole, 92% of patients showed upper or lower GI tract infiltration by MCL.


4. Colon involvement of MCL : lymphomatous polyposis type

ÁÂÃø »çÁøÀ» º¸½Ê½Ã¿À. Áö±Ý±îÁö Á¦°¡ º» Áõ·Ê Áß °¡Àå ÀüÇüÀûÀÎ lymphomatous polyposis ÇüÅÂÀÇ mantle cell lymphomaÀÔ´Ï´Ù.

2016-9-23 ³»½Ã°æ Áý´ãȸ ¼­¿ï´ëº´¿ø Áõ·Ê. ´ëÀå ¿ëÁ¾ EMR ÈÄ cyclin D1 (+) mantle cell lymphoma·Î Áø´ÜµÇ¾úÀ¸³ª ¼ö ³â °£ follow up loss°¡ µÇ¾úÀ½. ¼ö ³â ÈÄ ÃßÀû³»½Ã°æ¿¡¼­ SMT À¯»çÇÑ lymphomatous polyposis ¾ç»óÀ̾úÀ½. ù ÀÓ»ó»óÀÌ ´ëÀå ¿ëÁ¾À̾ú°í 10³â ÈıîÁö ÁÖµÈ º´¼Ò°¡ ´ëÀå°ú mesentery¿´±â ¶§¹®¿¡ primary colonic mantle cell lymphoma·Î ÆÇ´ÜÇÏ¿´À½.

8³âÀü Áø´ÜµÇ¾ú°í Ç×¾ÏÄ¡·á ÈÄ È£ÀüµÇ¾ú´Ù°¡ ÃÖ±Ù Àç¹ßµÊ. ¿ëÁ¾ ºÎÀ§Á¶Á÷°Ë»ç¿¡¼­ ¸ðµÎ mantle cell lymphoma°¡ ³ª¿È.


5. Colon involvement of MCL : non-lymphomatous polyposis type

üÁß°¨¼Ò¸¦ ÁÖ¼Ò·Î ³»¿øÇÑ 60´ë ¿©¼ºÀÇ ´ëÀå³»½Ã°æÀÔ´Ï´Ù. Á¶Á÷°Ë»ç¿¡¼­ mantle cell lymphoma, cyclin D1 (+)°¡ ³ª¿Ô½À´Ï´Ù. CT¿¡¼­ ileumÀÌ µÎ²¨¿ö º¸¿´½À´Ï´Ù. °æºÎ ¸²ÇÁÀý°ú °ñ¼ö ħÀ±ÀÌ ÀÖ¾ú½À´Ï´Ù.


6. Stomach involvement of MCL


¸í¶õÁ£Ã³·³ »ý°Ü¼­ ±ô¦ ³î¶ú½À´Ï´Ù.

9³â Àü tonsilÀÇ mantle cell lymphoma·Î chemotherapy + RT ¹ÞÀº ȯÀÚÀÇ À§ ³»½Ã°æÀÔ´Ï´Ù. Á¶Á÷°Ë»ç¿¡¼­ mantle cell lymphoma°¡ ³ª¿Í stomach RT¸¦ ½ÃÇàÇÏ¿´½À´Ï´Ù.


7. Pathology of MCL

1) MCL is characterized by neoplastic expansion of the mantle zone surrounding lymph node germinal center, with a homogeneous population of small lymphoid cells displaying slightly to markedly

2) irregular nuclear outlines, small nucleoili and scant cytoplasm.

3) In contrast to follicular lymphomas, very few large cells are identified among the mantle cells. The malignant cells are intermediate in size, between those of small lymphocytic lymphoma and

4) hose of follicular small cleaved cell lymphoma.

5) Small cell (small lymphocyte-like; intermediate lymphocyte) 80% of cases

6) ¡°Blastoid¡± (classical/monomorphic; pleomorphic) 20% of cases

7) B marker: IgM (+) or IgD(+), CD19, CD20, CD22, CD79

8) T marker: CD10(-), CD23 (-), CD5 (weakly +)

9) Cyclin D1 (+)

10) BCL6 negative


[Genetics of MCL]

1) t(11;14)(q13;q32)

2) Immunoglobulin (H and L chains) clonal rearrangement

3) IgVH unmutated (70-80% of cases) origin from a subset of (CD5+) ¡°naive¡± (pre-germinal centre) B lymphocytes

4) Conventional cytogenetics : often complex caryotype; del 6q, del 11q22, del 13q14¡¦

5) CGH : chromosomal gains (3q26-27,¡¦); and losses (13,Y,¡¦)


8. Cyclin D negative mantle cell lymphoma

Cyclin D negative MCL¸¦ Áø´ÜÇϱⰡ ¾î·Æ½À´Ï´Ù. ºóµµ´Â 2%·Î ÃßÁ¤µË´Ï´Ù. Cyclin D negativeÀÎ °æ¿ì SOX-11 expressionÀÌ Áø´ÜÇϴµ¥ µµ¿òµÉ ¼ö ÀÖ°í (Hematologica 2009. SOX11 mRNA and nuclear protein expression is a highly specific marker for both cyclin D1-positive and negative mantle cell lymphoma), SOX-11 expressionÀÌ À½¼ºÀÎ °æ¿ì indolentÇÑ °æ°ú¸¦ º¸ÀÌ´Â °æÇâÀÌ ÀÖ½À´Ï´Ù (Am J Hematol 2017 ).

(M/70, 2018)
À§Ã¼ºÎ, À§ÀüÁ¤ºÎ, ½ÊÀÌÁöÀå Á¶Á÷°Ë»ç ¸ðµÎ mantle cell lymphoma·Î ³ª¿Ô½À´Ï´Ù. Áø´Ü 3°³¿ù ÈÄ »ç¸ÁÇϼ̽À´Ï´Ù.
Diagnosis: B CELL LYMPHOMA with SOX11, PAX5 and CD138-positivity, suggestive of MANTLE CELL LYMPHOMA with plasma cell component
Ki-67 : Positive in 60% of tumor cells
CD 3, T-CELL : Negative in tumor cells
CD 20, L26 : Negative in tumor cells
CD 21, B-CELL : No follicular dendritic cell meshwork
SOX11 : Positive in tumor cells
CD 79a : Negative in tumor cells
Cyclin D-1 : Negative in tumor cells
Tdt : Negative in tumor cells
PAX-5 (Pan b Cell) : Weak positive in tumor cells
S-100 protein : Negative in tumor cells
CD 138, Syndecan-1 : Positive in tumor cells
Epstein-Barr virus: Negative in tumor cells


9. GI mantle cell lymphoma, experiences at Samsung Medical Center (Kim JH. Acta Haematol 2012)

Endoscopic findings in mantle cell lymphoma with gastrointestinal tract involvement.

BACKGROUND AND AIMS: Mantle cell lymphoma (MCL) of the gastrointestinal (GI) tract is a rare disease with a poor prognosis. The aim of this study was to determine clinical and endoscopic characteristics of patients with GI MCL.

METHODS: Clinical features of 19 patients with GI MCL were reviewed along with the endoscopic findings on 27 anatomical lesions.

RESULTS: The initial presenting symptoms were abdominal pain (n = 7, 36.8%), GI tract bleeding (n = 5, 26.3%), dyspnea (n = 2, 10.5%), indigestion (n = 1, 5.3%), diarrhea (n = 1, 5.3%), cervical lymphadenopathy (n = 1, 5.3%), tonsilar mass (n = 1, 5.3%), and no symptoms (n = 1, 5.3%). On endoscopy, in 19 patients with 27 lesions, the anatomic locations of the lesions were: stomach, n = 2 (10.5%); stomach and colon, n = 7 (36.8%); terminal ileum and colon, n = 1 (5.3%); colon, n = 9 (47.4%). There was 1 fungating case (3.7%), 4 ulcerative cases (14.8%), 9 infiltrative cases (33.3%), and 13 polypoid cases (48.1%).

CONCLUSIONS: The endoscopic findings in GI MCL are variable, with common presenting manifestations of abdominal pain and GI bleeding.



© ÀÏ¿ø³»½Ã°æ±³½Ç ¹Ù¸¥³»½Ã°æ¿¬±¸¼Ò ÀÌÁØÇà. EndoTODAY Endoscopy Learning Center. Lee Jun Haeng.