B lymphoblastic leukemia/lymphoma with hypodiploidy (Hypodiploid ALL)

Name
B lymphoblastic leukemia/lymphoma with hypodiploidy (Hypodiploid ALL)
ICD-O-3 Morphology
9816/3
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. Lymphomatous presentation is rare.

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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 whose blasts contain <46 chromosomes. A stricter definition of <45 chromosomes, and possibly even <44 chromosomes more accurately reflects the clinical pathologic entity.

This disease is seen in both children and adults, although near haploid ALL (23-29 chromosomes) appears limited to childhood.
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].)

Hypodiploid ALL has a poor prognosis. The prognosis for this disease depends on the number of chromosomes; those with 44-45 chromosomes have the best prognosis. Patients may fare poorly even if they do not have residual disease following therapy.
Definitive Diagnostic Methods
Bone marrow biopsy
FISH
Flow cytometry
Genetic testing
Immunophenotyping
Karyotyping
Genetics Data
Loss of one or more chromosomes
Immunophenotyping
CD10+
CD19+
Treatments
Chemotherapy
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
Hepatomegaly
Lymphoadenopathy
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: limited to childhood (although can be seen in adulthood)
Incidence: 5% of cases of ALL
Survival: poor prognosis