Name
ICD-O-3 Morphology
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.
Coding Manual:
Hematopoietic Coding Manual (PDF)
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 presenting features are generally similar to those of other patients with ALL. Cases of T-ALL should not be included in this code even though those patients have near tetraploid karyotypes.
Immunophenotyping will confirm that the patient has a B-lymphoblastic B-ALL; the genetic testing provides the information needed to classify this neoplasm as a B lymphoblastic leukemia/lymphoma with hyperdiploidy.
The presenting features are generally similar to those of other patients with ALL. Cases of T-ALL should not be included in this code even though those patients have near tetraploid karyotypes.
Immunophenotyping will confirm that the patient has a B-lymphoblastic B-ALL; the genetic testing provides the information needed to classify this neoplasm as a B lymphoblastic leukemia/lymphoma with hyperdiploidy.
Diagnostic Confirmation
This histology can only be determined by positive genetics and/or immunophenotyping, diagnostic confirmation will always be 3.
Grade
Not Applicable
Module Rule
See abstractor notes
Alternate Names
B-ALL/LBL with hyperdiploidy
Definition
B-lymphoblastic leukemia/lymphoma with hyperdiploidy is a neoplasm of lymphoblasts committed to the B-cell lineage whose blasts contain > 50 chromosomes (usually < 66), typically without translocations or other structural alterations.
Definitive Diagnostic Methods
Bone marrow biopsy
FISH
Genetic testing
Immunophenotyping
Karyotyping
Genetics Data
Extra copies of chromosomes 21, X, 14 and 4
Immunophenotyping
CD10+ (expression/positive)
CD19+ (expression/positive)
CD34+ (expression/positive)
CD45- (no expression/negative)
Treatments
Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Transformations to
There are no known transformations
Transformations from
There are no known transformations
Same Primaries
Corresponding ICD-9 Codes
200.1 Lymphosarcoma (Lymphoma presentation)
204.0 Acute lymphoid leukemia (Leukemia presentation)
Corresponding ICD-10 Codes
C83.5 Non-Hodgkin lymphoma lymphoblastic (diffuse) (Lymphoma presentation)
C91.0 Acute lymphoblastic leukemia (Leukemia presentation)
Corresponding ICD-10-CM Codes (U.S. only)
C83.5 Lymphoblastic (diffuse) lymphoma (Lymphoma presentation) (effective October 01, 2015)
C91.0 Acute lymphoblastic leukemia [ALL] (Leukemia presentation) (effective October 01, 2015)
Signs and Symptoms
Anemia
Arthralgias
Bone pain
Hepatomegaly
Lymphoadenopathy
Neutropenia
Splenomegaly
Thrombocytopenia
Diagnostic Exams
Blood chemistry studies
Bone marrow aspiration and biopsy
CT (CAT) scan
Cytogenetic analysis
Flow cytometry
Immunohistochemistry
Immunophenotyping
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
Lymph node biopsy
PET (positron emission tomography) scan
Progression and Transformation
None
Epidemiology and Mortality
Sources
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Precursor lymphoid neoplasms
Pages: 205-206
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Precursor lymphoid neoplasms
Pages: 205-206
International Classification of Diseases for Oncology, Third Edition, Second Revision. Geneva: World Health Organization, 2020.
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577
National Cancer Institute
Section: General Information About Acute Lymphoid Leukemia
Pages: https://www.cancer.gov/types/leukemia/patient/adult-all-treatment-pdq
Section: General Information About Acute Lymphoid Leukemia
Pages: https://www.cancer.gov/types/leukemia/patient/adult-all-treatment-pdq