Precursor T-cell acute lymphoblastic…

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Precursor T-cell acute lymphoblastic leukemia

ORPHA:99861Disease

Also called Precursor T-cell acute lymphoblastic leukemia/lymphoma · Precursor T-cell acute lymphocytic leukemia · Precursor T-cell acute lymphocytic leukemia/lymphoma · T-ALL

What it is

A rare acute lymphoblastic leukemia characterized by a neoplasm of lymphoblasts committed to the T-cell lineage, involving bone marrow and blood. A value of >25% bone marrow blasts may be used to define leukemia (as opposed to lymphoma) in cases with the presence of a mass lesion in addition to bone marrow involvement. Patients typically present with leukocytosis, and frequently with a large mediastinal or other tissue mass. Lymphadenopathy and hepatosplenomegaly are common.

Key facts

Age of onset
Adolescent, Adult
Inheritance
Not applicable
Classified as
Disease

Recorded for the broader condition

Prevalence
1-5 / 10 000 (Europe)Acute lymphoblastic leukemia

Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.

Genes

CDKN2ADisease-causing somatic mutation(s)
CNOT3Disease-causing somatic mutation(s)
FLT3Disease-causing somatic mutation(s)
ABL1Part of a fusion gene
BCRPart of a fusion gene
DDX3XPart of a fusion gene
MLLT10Part of a fusion gene
MYBPart of a fusion gene
MYCPart of a fusion gene
NUP214Part of a fusion gene
PICALMPart of a fusion gene
SALL2Part of a fusion gene
SETPart of a fusion gene
STILPart of a fusion gene
TAL1Part of a fusion gene
TCL1APart of a fusion gene
TLX1Part of a fusion gene
TLX3Part of a fusion gene
TRAPart of a fusion gene
TRBPart of a fusion gene
TRDPart of a fusion gene
ZBTB16Part of a fusion gene

ICD-10 codes

C91.0filed under a broader ICD-10 category — shared with 9 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

MESH D054218MONDO 0020512UMLS C1961099

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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