No Adverse Effect of Residual Neoplastic Mast Cells in Systemic Mastocytosis Associated with Acute Myeloid Leukemia with T(8;21)(Q22;Q22); Runx1-Runx1t1 after Bone Marrow Transplantation

نویسندگان

  • Zhao Ming Dong
  • Aravind Ramakrishnan
  • Thomas R Chauncey
  • Michael R. Loken
  • Barbara Zehentner
  • William H Schubach
چکیده

Mast cells are often increased in acute myeloid leukemia (AML) with t(8;21), however, concurrent development of systemic mastocytosis (SM) is rare. Here we report the case of a 22-year-old man with concurrent AML and SM. Although he achieved remission with chemotherapy, he relapsed with development of a myeloid sarcoma in the brain. After successful matched sibling hematopoietic stem cell transplant, neoplastic mast cells carrying both the RUNX1-RUNX1T1 translocation and the c-kit point mutation D816 V persisted in the marrow for up to 18 months after transplantation. Despite this, leukemia remains in continuous complete remission for 8.75 years. This observation suggests that neoplastic mast cells in SM-AML with t(8;21) have no adverse effect on hematopoiesis after bone marrow transplantation.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Systemic mastocytosis associated with t(8;21)(q22;q22) acute myeloid leukemia

Although KIT mutations are present in 20-25% of cases of t(8;21)(q22;q22) acute myeloid leukemia (AML), concurrent development of systemic mastocytosis (SM) is exceedingly rare. We examined the clinicopathologic features of SM associated with t(8;21)(q22;q22) AML in ten patients (six from our institutions and four from published literature) with t(8;21) AML and SM. In the majority of these case...

متن کامل

Duplication of der(21)t(8;21)(q22;q22) in acute myeloid leukemia.

The t(8;21)(q22;q22) results in the formation of RUNX1/ RUNX1T1 (alias AML1/ETO) and RUNX1T1/RUNX1 fusion genes on der(8)t(8;21)(q22;q22) and der(21)t(8;21)(q22;q 22), respectively. An 18-year-old woman was diagnosed as having acute myeloid leukemia (AML) M2, as her bone marrow was infiltrated with 42.2% myeloblasts. Auer rods were found in myeloblasts and metamyelocytes (Picture A, B, arrows)....

متن کامل

Coexistent t(8;21)(q22;q22) Translocation and 5q Deletion in Acute Myeloid Leukemia.

The t(8;21)(q22;q22) translocation is specifically observed in acute myeloid leukemia (AML) M2 subtype, whereas del(5q) is one of the most common cytogenetic aberrations in myelodysplastic syndromes (MDS). Thus, t(8;21)(q22;q22) and del(5q) appear to be mutually exclusive, and the association between them has not been characterized yet. Here, we report an 81-year-old woman with coexistent t(8;2...

متن کامل

A Case of Systemic Mastocytosis Associated with Acute Myeloid Leukemia Terminating as Aleukemic Mast Cell Leukemia after Allogeneic Hematopoietic Stem Cell Transplantation

In up to 40% of systemic mastocytosis (SM) cases, an associated clonal hematological non-mast cell lineage disease such as AML is diagnosed before, simultaneously with, or after the diagnosis of SM. A 40-yr-old man was diagnosed with AML with t(8;21)(q22;q22). Mast cells were not noted at diagnosis, but appeared as immature forms at relapse. After allogeneic hematopoietic stem cell transplantat...

متن کامل

A new complex translocation t(8;11;21)(q22;q24;q22) in acute myeloid leukemia with RUNX1/RUNX1T1.

The t(8;21)(q22;q22) translocation involving RUNX1 at 21q22 and RUNX1T1 at 8q22 is found in 10% of cases of acute myeloid leukemia (AML) M2 subtype. This translocation results in the formation of a RUNX1/RUNX1T1 fusion gene, which contributes to leukemic transformation by transcriptional repression of normal RUNX1 target genes, on der (8)t(8;21)(q22;q22). AML with t(8;21) is usually associated ...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:

دوره   شماره 

صفحات  -

تاریخ انتشار 2014