Main features of MCL cases with a previous history of pediatric mastocytosis
| Author . | Year . | Sex . | Age, mo* . | Type† . | Clinical evolution . | MC phenotype . | KIT sequencing . | Cytological features . | Treatment . |
|---|---|---|---|---|---|---|---|---|---|
| Waters37 | 1957 | M | 9 | DCM | 5 y: MCL transformation | ND | ND | Mast cells appeared as large polymorphic cells with acidophilic cytoplasm and large rounded nucleus, few metachromatic granules were discerned in the cytoplasm. | None |
| Death within 3 mo due to diffuse hemorrhage. | |||||||||
| Akin31 | 2004 | F | 5 | MPCM | 22 y: deterioration of general status | CD2neg | Phe 522 Cys | Round, highly granulated and centrally located nucleus | IFNα: no response |
| 25 y: ASM | CD25neg | (exon 10) | Imatinib: PR | ||||||
| And 6 mo later: MCL | FU: 5 mo | ||||||||
| Mital30 | 2011 | F | 12 | MPCM | 47 y: unexplained splenomegaly | CD2low | 502-503 Dup | Large mast cells | 2CDA: no response |
| 53 y: aleukemic MCL | CD25low | (exon 9) | Imatinib: PR | ||||||
| 56 y: MCL evolution | FU: 18 mo | ||||||||
| Chantorn36 | 2012 | F | 5 | Mastocytoma | 10 y: MPCM | CD2− | ND | Rare spindle shaped mast cells on bone marrow histology | poly chemotherapy, |
| 19 y: mastocytoma exeresis | CD25+ | Atypically spindle shaped morphology on BM smear | allo-SCT, Steroids | ||||||
| 23 y: ASM; MCL and death 5 mo later | Tacrolimus: | ||||||||
| No response |
| Author . | Year . | Sex . | Age, mo* . | Type† . | Clinical evolution . | MC phenotype . | KIT sequencing . | Cytological features . | Treatment . |
|---|---|---|---|---|---|---|---|---|---|
| Waters37 | 1957 | M | 9 | DCM | 5 y: MCL transformation | ND | ND | Mast cells appeared as large polymorphic cells with acidophilic cytoplasm and large rounded nucleus, few metachromatic granules were discerned in the cytoplasm. | None |
| Death within 3 mo due to diffuse hemorrhage. | |||||||||
| Akin31 | 2004 | F | 5 | MPCM | 22 y: deterioration of general status | CD2neg | Phe 522 Cys | Round, highly granulated and centrally located nucleus | IFNα: no response |
| 25 y: ASM | CD25neg | (exon 10) | Imatinib: PR | ||||||
| And 6 mo later: MCL | FU: 5 mo | ||||||||
| Mital30 | 2011 | F | 12 | MPCM | 47 y: unexplained splenomegaly | CD2low | 502-503 Dup | Large mast cells | 2CDA: no response |
| 53 y: aleukemic MCL | CD25low | (exon 9) | Imatinib: PR | ||||||
| 56 y: MCL evolution | FU: 18 mo | ||||||||
| Chantorn36 | 2012 | F | 5 | Mastocytoma | 10 y: MPCM | CD2− | ND | Rare spindle shaped mast cells on bone marrow histology | poly chemotherapy, |
| 19 y: mastocytoma exeresis | CD25+ | Atypically spindle shaped morphology on BM smear | allo-SCT, Steroids | ||||||
| 23 y: ASM; MCL and death 5 mo later | Tacrolimus: | ||||||||
| No response |
MCL indicates mast cell leukemia; M, male; DCM, diffuse cutaneous leukemia; ND, not done; F, female; MPCM, maculopapular cutaneous mastocytosis; ASM, aggressive systemic mastocytosis; IFNa, interferon alpha; PR, partial response; FU, follow-up; BM, bone marrow; and allo-SCT, allogeneic stem cell transplantation.
Age at diagnosis of pediatric mastocytosis.
Type of pediatric mastocytosis at diagnosis.