B lymphoblastic leukemia/lymphoma with t(v;11q23); MLL rearranged

Name
B lymphoblastic leukemia/lymphoma with t(v;11q23); MLL rearranged
ICD-O-3 Morphology
9813/3
Effective 2010 and later
Reportable
for cases diagnosed 2010 and later
Primary Site(s)
See Module 4: Rules PH7, PH8
Usually presents as a leukemia. Organ involvement is possible. Lymphomatous presentation is rare. High incidence of CNS involvement at diagnosis

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Grade
6 - B-cell
Module Rule
Module 4: PH7, PH8
Alternate Names
None
Definition
A neoplasm of lymphoblasts committed to the B lineage that harbours a translocation between the MLL gene at band 11q23 and any one of a large number of different fusion partners.

Patients with leukemias that have deletions of 11q23 without MLL rearrangements are not included in this group.
Abstractor Notes
(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010, see codes: 9728/3 [lymphoma] or 9836/3 [leukemia].)

The specific etiology of leukemia with MLL translocations is unknown; however, it may occur in utero, with a short latency between the translocation and development of disease. Evidence for this statement includes the fact that these leukemias are frequent in very young infants.
Definitive Diagnostic Methods
Bone marrow biopsy
Genetic testing
Immunophenotyping
Genetics Data
AF4 on chromosome 4q21
AF9 on chromosome 9p22
ENL on chromosome 19p13
FLT-3
MLL gene on chromosome 11q23
MLL-ENL
Immunophenotyping
CD10-
CD15+
CD19+
CD24-negative pro-B immunophenotype
Chondroitin sulfate proteo-glycan neural-glial antigen
Treatments
Chemotherapy
Stem cell transplant
Transformations to
There are no known transformations
Transformations from
There are no known transformations
Corresponding ICD-9 Codes
204.8 Other lymphoid leukemia
Corresponding ICD-10 Codes
C91.7 Other lymphoid leukemia
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C91.Z Other lymphoid leukemia
Signs and Symptoms
Anemia
Arthralgias
Bone pain
Elevated white blood cell (WBC) count
Hepatomegaly
Lymphadenopathy
Splenomegaly
Diagnostic Exams
Bone marrow aspiration and biopsy
CT (CAT) scan
Complete blood count (CBC)
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
MRI (magnetic resonance imaging)
PET (positron emission tomography) scan
Progression and Transformation
None
Epidemiology and Mortality
Age: may occur in utero, frequently found in young infants
Incidence: most common leukemia in infants <1 year of age
Survival: poor prognosis (especially for infants less than 6 months of age)