Clinical and biologic characteristics of MCL patients: literature review
| . | MCL . | MCL with pediatric mastocytosis, n = 4 . | ||
|---|---|---|---|---|
| All, N = 51 . | De novo, n = 30 . | Secondary, n = 11 . | ||
| Median age at diagnosis, y (range) | 52 (5-76) | 51.5 (18-76) | 35 (5-75) | 24 (5-53) |
| Female/Male | 30/20 | 18/11 | 5/6 | 3/1 |
| Ethnicity, n (%) | ||||
| White | 18/21 (86) | 13/15 (87) | 5/6 (83) | 2 (50) |
| Asiatic | 3/21 (14) | 2/15 (13) | 1/6 (16) | 1 (25) |
| Familial mastocytosis, n (%) | 0 (0) | 0 (0) | 0 (0) | 0 (0) |
| Median time from symptoms to diagnosis, mo (range) | 5 (0-108) | 3 (0-24) | 6 (0-108) | 4.5 (0-108) |
| History of mastocytosis, n (%) | 11/40 (27) | 11 (100) | 4 (100) | |
| Aleukemic MCL, n (%) | 29/47 (62) | 15 (50) | 7 (70) | 4 (100) |
| Organ involvement, n (%) | ||||
| Skin, UP | 15/50 (30) | 4 (14) | 5 (45) | 4 (100) |
| Hepatomegaly | 32/47 (68) | 21 (78) | 6 (60) | 2 (50) |
| Splenomegaly | 38/49 (65) | 23 (82) | 9 (82) | 4 (100) |
| Lymph nodes | 16/43 (37) | 11 (44) | 3 (33) | 1/2 (50) |
| Ascites | 9/50 (18) | 5 (17) | 1 (9) | 0 (0) |
| Gastroduodenal ulcer, n (%) | 11/38 (29) | 10 (38.4) | 0 (0) | 0 (0) |
| AHNMD, n (%) | 4/40 (10) | 3 (11) | 0 | 0 |
| MCAS, n (%) | 39/50 (78) | 24 (83) | 8 (73) | 3 (75) |
| KITmutation | 28/51 (55) | 15 (50) | 5 (45) | 2 (50) |
| D816V KIT mutation | 13/28 (46) | 6 (40) | 1 (20) | 0 |
| WT KIT | 2/28 (7) | 1 (6) | 1 (20) | 0 |
| 502-503 KIT mutation | 3/28 (11) | 2 (13) | 1 (20) | 1 |
| 522/560/654 KIT mutation | 3/28 (11) | 1 (6) | 2 (40) | 1 |
| Abnormal cytogenetics, n (%)* | 4/24 (17) | 3 (27) | ||
| Phenotype, n (%) | ||||
| CD2+ | 15/29 (52) | 6 (46) | 1 | 0 |
| CD25+ | 18/24 (75) | 6 (50) | 3 | 1/3 |
| CD2−/CD25+ | 3/24 | 1 | 1 | 1/3 |
| CD2−/CD25− | 9/24 | 5 | 3 | 2/3 |
| CD2+/CD25+ | 13/24 | 4 | 1 | 0 |
| CD2+/CD25− | 1/24 | 1 | 0 | 0 |
| Laboratory data, median (range) | ||||
| Tryptase, ng/L | 433 (21-2357) | 433 (21-742) | 250 (173-2357) | 173.5 (173-200) |
| Hemoglobin, g/dL | 9.9 (5.4-14) | 9 (5.4-13.7) | 11 (8.1-13.3) | 12.5 (8.1-13.3) |
| Platelets, G/L | 110 (5-318) | 82 (5-202) | 111 (30-150) | 136 (101-318) |
| PMN, G/L | 3.65 (0.99-15.3) | 6.0 (0.99-14) | 8.5 (1.7-15.3) | |
| BM mastocytes % | 50 (20-100) | 60 (20-100) | 60 (25-90) | 60 (25-75) |
| Blood mastocytes % | 5.5 (0-72) | 7 (0-72) | 0 (0-12) | 2 (one tested) |
| Outcome | ||||
| Death, n (%) | 33/48 (69) | 24 (83) | 5 (62) | 2 (50%)† |
| Median survival time, mo (range) | 6 (0.5-98) | 4 (0.5-24) | 5 (1-18) | |
| . | MCL . | MCL with pediatric mastocytosis, n = 4 . | ||
|---|---|---|---|---|
| All, N = 51 . | De novo, n = 30 . | Secondary, n = 11 . | ||
| Median age at diagnosis, y (range) | 52 (5-76) | 51.5 (18-76) | 35 (5-75) | 24 (5-53) |
| Female/Male | 30/20 | 18/11 | 5/6 | 3/1 |
| Ethnicity, n (%) | ||||
| White | 18/21 (86) | 13/15 (87) | 5/6 (83) | 2 (50) |
| Asiatic | 3/21 (14) | 2/15 (13) | 1/6 (16) | 1 (25) |
| Familial mastocytosis, n (%) | 0 (0) | 0 (0) | 0 (0) | 0 (0) |
| Median time from symptoms to diagnosis, mo (range) | 5 (0-108) | 3 (0-24) | 6 (0-108) | 4.5 (0-108) |
| History of mastocytosis, n (%) | 11/40 (27) | 11 (100) | 4 (100) | |
| Aleukemic MCL, n (%) | 29/47 (62) | 15 (50) | 7 (70) | 4 (100) |
| Organ involvement, n (%) | ||||
| Skin, UP | 15/50 (30) | 4 (14) | 5 (45) | 4 (100) |
| Hepatomegaly | 32/47 (68) | 21 (78) | 6 (60) | 2 (50) |
| Splenomegaly | 38/49 (65) | 23 (82) | 9 (82) | 4 (100) |
| Lymph nodes | 16/43 (37) | 11 (44) | 3 (33) | 1/2 (50) |
| Ascites | 9/50 (18) | 5 (17) | 1 (9) | 0 (0) |
| Gastroduodenal ulcer, n (%) | 11/38 (29) | 10 (38.4) | 0 (0) | 0 (0) |
| AHNMD, n (%) | 4/40 (10) | 3 (11) | 0 | 0 |
| MCAS, n (%) | 39/50 (78) | 24 (83) | 8 (73) | 3 (75) |
| KITmutation | 28/51 (55) | 15 (50) | 5 (45) | 2 (50) |
| D816V KIT mutation | 13/28 (46) | 6 (40) | 1 (20) | 0 |
| WT KIT | 2/28 (7) | 1 (6) | 1 (20) | 0 |
| 502-503 KIT mutation | 3/28 (11) | 2 (13) | 1 (20) | 1 |
| 522/560/654 KIT mutation | 3/28 (11) | 1 (6) | 2 (40) | 1 |
| Abnormal cytogenetics, n (%)* | 4/24 (17) | 3 (27) | ||
| Phenotype, n (%) | ||||
| CD2+ | 15/29 (52) | 6 (46) | 1 | 0 |
| CD25+ | 18/24 (75) | 6 (50) | 3 | 1/3 |
| CD2−/CD25+ | 3/24 | 1 | 1 | 1/3 |
| CD2−/CD25− | 9/24 | 5 | 3 | 2/3 |
| CD2+/CD25+ | 13/24 | 4 | 1 | 0 |
| CD2+/CD25− | 1/24 | 1 | 0 | 0 |
| Laboratory data, median (range) | ||||
| Tryptase, ng/L | 433 (21-2357) | 433 (21-742) | 250 (173-2357) | 173.5 (173-200) |
| Hemoglobin, g/dL | 9.9 (5.4-14) | 9 (5.4-13.7) | 11 (8.1-13.3) | 12.5 (8.1-13.3) |
| Platelets, G/L | 110 (5-318) | 82 (5-202) | 111 (30-150) | 136 (101-318) |
| PMN, G/L | 3.65 (0.99-15.3) | 6.0 (0.99-14) | 8.5 (1.7-15.3) | |
| BM mastocytes % | 50 (20-100) | 60 (20-100) | 60 (25-90) | 60 (25-75) |
| Blood mastocytes % | 5.5 (0-72) | 7 (0-72) | 0 (0-12) | 2 (one tested) |
| Outcome | ||||
| Death, n (%) | 33/48 (69) | 24 (83) | 5 (62) | 2 (50%)† |
| Median survival time, mo (range) | 6 (0.5-98) | 4 (0.5-24) | 5 (1-18) | |
MCL indicates mast cell leukemia; UP, urticaria pigmentosa; AHNMD, associated clonal hematologic non–MC-lineage disease; MCAS, mast cell activation syndrome including flushes, fever, malaise, diarrhea, and tachycardia; WT, wild type; PMN, polymorphonuclear; and BM, bone marrow.
Abnormal cytogenetics, 5q deletion (2 patients with AHNMD), t(10;16) (q22;q13q22), and t(8;21)(q22;q22) (2 patients).
No information on the other 2 patients at 4 months.