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 Module 4: Rules PH7, PH8
Most common sites of involvement: lymph nodes, peripheral blood

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
Mature T ALL
Pre-T ALL
Precursor T acute lymphoblastic leukemia
Precursor T-cell lymphoblastic leukemia
Pro-T ALL
T-ALL
T-LBL
Definition
Prolymphocytic leukemia, a chronic lymphocytic leukemia, is divided into two types according to the kind of cell involved:
1. B-cell prolymphocytic leukemia
2. T-cell prolymphocytic 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 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.
Definitive Diagnostic Methods
Bone marrow biopsy
Genetic testing
Histologic confirmation
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
Hepatosplenomegaly
High leukocyte count
Lymphadenopathy
Pleural effusions
Rapid growth mediastinal mass (anterior mediastinum)
Respiratory failure (emergency)
Diagnostic Exams
CT (CAT) scan
Complete blood count (CBC)
Cytogenetic analysis
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
MRI (magnetic resonance imaging)
PET (positron emission tomography) scan
Recurrence and Metastases
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