T lymphoblastic leukemia/lymphoma

Name
T lymphoblastic leukemia/lymphoma
ICD-O-3 Morphology
9837/3
Effective 2001 and later
Reportable
for cases diagnosed 2001 and later
Primary Site(s)
See Abstractor Notes and Module 4: Rules PH7, PH8

Help me code for diagnosis year :

Grade
5 - T-cell
Module Rule
Module 4: PH7, PH8
Alternate Names
Chronic T-cell leukemia/lymphoma
Cortical T ALL
Lymphomatous T-cell leukemia/lymphoma
Mature T ALL
Pre-T ALL
Precursor T acute lymphoblastic leukemia
Precursor T-cell lymphoblastic leukemia
Pro-T ALL
T-ALL
T-acute lymphoblastic leukemia
T-acute lymphoblastic lymphoma
T-cell leukemia/lymphoma
T-LBL
Definition
Prolymphocytic leukemia is divided into two types accourding to the kind of cell involved: B-cell prolymphocytic leukemia and T-cell prolymphocytic leukemia. It is usually classified as a kind of chronic lymphocytic leukemia.

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.
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 extralymphatic site of involvement. The disease is usually systemic, involving the spleen and extranodal sites including skin, lung, liver, GI tract and CNS. Epidemiological differences occur in patterns of presentation. For example, the peripheral blood involvement is much less in patients from the Carribean basin than from Japan. Several clinical variants have been identified: acute, lymphomatous, chronic, and smoldering ATLL.

T-ALL comprises about 15% of childhood ALL. It is more common in adolescents than in younger children and more common in males than females. T-ALL comprises approximately 25% of adult ALL. Patients often present with a high leukocyte count and often a large mediastinal mass or other tissue mass. Lymphadenopathy and hepatosplenomegaly are common.
Definitive Diagnostic Methods
Bone marrow biopsy
Genetics testing
Histology-tissue or node
Immunophenotyping
Karyotyping
Peripheral blood smear
Genetics Data
Alpha and Beta TCR loci at 14q11.2
Beta locus at 7q35
Gamma locus at 7p14-15
IGH@gene rearrangements
Rearrangement of T-cell receptor genes (TCR)
Immunophenotyping
ALK-
CCR4+
CD2+
CD3+
CD5+
CD7-
CD25 strongly expressed
FoxP3+
Treatments
Bone marrow transplant
Chemotherapy
Hormone
Radiation
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
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None