Bone marrow neoplastic

Bone marrow - plasma cell and lymphoid neoplasms

NK and T cell precursor lymphoid neoplasms

T lymphoblastic lymphoma / leukemia



Last author update: 1 March 2013
Last staff update: 5 October 2023

Copyright: 2001-2024, PathologyOutlines.com, Inc.

PubMed Search: T cell acute lymphoblastic leukemia / lymphoma

Page views in 2023: 11,259
Page views in 2024 to date: 1,643
Cite this page: Mihova D. T lymphoblastic lymphoma / leukemia. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/leukemiaTall.html. Accessed December 2nd, 2024.
Definition / general
  • Neoplasm of T lineage lymphoblasts which may form lymphomatous masses, involve blood and bone marrow (Stanford School of Medicine: Precursor T Lymphoblastic Leukemia / Lymphoma [Accessed 13 April 2018])
  • Also called pre T cell acute lymphocytic leukemia / lymphoma (pre-T ALL), T lymphoblastic leukemia / lymphoma (T LBL)
  • Teens and young men (older than B ALL)
  • Most cases begin after birth (Blood 2007;110:3036)
  • T ALL versus T LBL: T ALL has more immature phenotype, CD47 expression, no 11q23 rearrangement, different gene expression profile and may derive from T cell progenitor of bone marrow; T LBL is derived from thymocytes (Pediatr Blood Cancer 2006;47:130, Leuk Lymphoma 2007;48:1745)
  • T ALL constitutes 15% of childhood and 20 - 25% of adult ALL cases
  • T LBL constitutes 85 - 95% of LBL, usually presents as mediastinal mass with no / minimal marrow involvement
  • CNS involvement if untreated
  • T LBL frequently presents with mass in anterior mediastinum, rapid growth, respiratory emergency, pleural effusion
  • Younger (age 16 - 60 years) patients compared to older (61+ years) patients have more hepatosplenomegaly, present with mediastinal mass and lymphadenopathy; myeloid antigens and lineage inappropriate gene rearrangements are less common (Am J Clin Pathol 2002;117:252)
Diagrams / tables

Images hosted on other servers:
Missing Image

Rearrangements involving T cell receptor genes

Diagnosis
  • T ALL if lymphoblasts are 25% or more of marrow cells; T LBL otherwise
Prognostic factors
Treatment
  • Chemotherapy cures 60%
  • Patients have earlier relapse, induction failure and isolated CNS relapse compared to pre-B ALL
Microscopic (histologic) description
  • Similar to B cell disease; scant cytoplasm, delicate chromatin, indistinct nucleoli, convoluted nuclear membrane and grooves
  • Frequent mitotic figures; starry sky pattern produced by interspersed benign macrophages
  • Usually features of FAB L1 or L2; pattern in marrow is usually interstitial
  • Lymph nodes: complete architectural effacement or partial involvement with paracortical infiltrate with germinal center sparing
  • Thymus: replacement of normal parenchyma
  • Occasionally eosinophilia and myeloid hyperplasia with variable t(8;13)(p11.2;q11-22) involving FGFR1 gene; some develop myeloid malignancy (MDS, AML or myeloid sarcoma)
Microscopic (histologic) images

AFIP images
Missing Image

Bone marrow biopsy

Missing Image

Focal paranuclear acid phosphatase staining

Peripheral smear images

AFIP images
Missing Image

Markedly elevated leukocyte count

Missing Image

L2 type (blood smears)

Positive stains
Negative stains
  • CD19, CD20; double negative CD4 / CD8 (40%)
  • Note: ALL with aberrant myeloid antigen expression is correct name, not biphenotypic leukemia
Molecular / cytogenetics description

Genes:
  • MYC (8q24.1)
  • TAL1 (1p32)
  • RBTN1 (LMO1) (11p15)
  • RBTN2 (LMO2) (11p13)
  • HOX11 (TLX1) (10q24)
  • HOX11L2 (TLX3) (5q35)
  • LYL1 (19p13)
  • LCK (1p34.3-35)
Molecular / cytogenetics images

Images hosted on other servers:
Missing Image

FISH: t(5;14)(q35;q32)

Differential diagnosis
Back to top
Image 01 Image 02