Reportable
for cases diagnosed
2001 and later
Primary Site(s)
See Module 4: Rules PH7, PH8
Most common sites of involvement: lymph nodes, peripheral blood
Coding Manual:
Hematopoietic Coding Manual (PDF)
Abstractor Notes
Most ALL patients present with widespread lymph node involvement as well as peripheral blood involvement. The number of circulating neoplastic cells does not correlate with the degree of bone marrow involvement, suggesting that circulating cells are recruited from other organs such as the skin. In fact, the skin is the most common extra-lymphatic site of involvement.
The disease is usually systemic, involving the spleen and extranodal sites including skin, lung, liver, GI tract and CNS.
Several clinical variants have been identified:
1. Acute
2. Chronic
3. Lymphomatous
4. Smoldering ATLL
Patients often present with a high leukocyte count and often a large mediastinal mass or other tissue mass.
T-ALL comprises approximately 25% of adult ALL.
The disease is usually systemic, involving the spleen and extranodal sites including skin, lung, liver, GI tract and CNS.
Several clinical variants have been identified:
1. Acute
2. Chronic
3. Lymphomatous
4. Smoldering ATLL
Patients often present with a high leukocyte count and often a large mediastinal mass or other tissue mass.
T-ALL comprises approximately 25% of adult ALL.
Diagnostic Confirmation
This histology can be determined by positive histology (including peripheral blood) with or without genetics and/or immunophenotyping. Review the Definitive Diagnostic Methods, Immunophenotyping and Genetics Data sections below, and the instructions in the Hematopoietic Manual for further guidance on assigning Diagnostic confirmation.
Grade
Not Applicable
Module Rule
Module 4: PH7, PH8
Definition
T-lymphoblastic leukemia/lymphoma (T-ALL) is a neoplasm of lymphoblasts committed to the T-cell lineage, typically composed of small to medium-sized blast cells with scant cytoplasm, moderately condensed to dispersed chromatin, and inconspicuous nucleoli, involving bone marrow and blood (T-ALL) or presenting with primary involvement of the thymus or of nodal or extranodal sites (T-LBL).
Definitive Diagnostic Methods
Bone marrow biopsy
Genetic testing
Histologic confirmation
Immunophenotyping
Karyotyping
Peripheral blood smear
Genetics Data
Immunophenotyping
CCR4+ (expression/positive)
CD1a+ (expression/positive)
CD2+ (expression/positive)
CD3+ (expression/positive)
CD4+ (expression/positive)
CD5+ (expression/positive)
CD7+ (expression/positive)
CD8+ (expression/positive)
C13+ (expression/positive)
CD19+ (expression/positive)
CD25+ (expression/positive)
CD33+ (expression/positive)
CD79a+ (expression/positive)
FLT3 mutation
KIT (CD117)+ (expression/positive)
Treatments
Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Hormone therapy
Radiation therapy
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
Hepatosplenomegaly
High leukocyte count
Lymphadenopathy
Pleural effusions
Rapid growth mediastinal mass (anterior mediastinum)
Respiratory failure (emergency)
Diagnostic Exams
Blood chemistry studies
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
Age: more common in adolescents than younger children and adults
Country: Patients from the Carribean basin than from Japan usually don't have peripheral blood involvement
Incidence: ~15% of childhood ALL
Sex: slight female predominance
Survival: higher risk disease than B-ALL
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: 209-212
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Precursor lymphoid neoplasms
Pages: 209-212
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