Name

Myeloid/lymphoid neoplasms with PCM1-JAK2

ICD-O-3 Morphology

9968/3: Myeloid/lymphoid neoplasms with PCM1-JAK2
Effective 2021 and later

Reportable

for cases diagnosed 2021 and later

Primary Site(s)

C421
Primary site must be bone marrow (C421). Blood and bone marrow always involved.

Abstractor Notes

(This code is effective for cases diagnosed 2021 and later. For cases diagnosed prior to 2021 see code: 9975/3.)

The peripheral blood and bone marrow are involved. Patients often have hepatosplenomegaly.

Grade

Not Applicable

Module Rule

None

Alternate Names

None

Definition

Myeloid/lymphoid neoplasms associated with t(8;9)(p22;p24.1) and PCM1-JAK2 share characteristic features that justify the recognition of the group as a provisional entity.

The hematological features may be those characteristic of a myeloproliferative neoplasm or those characteristic of a myelodysplastic/myeloproliferative neoplasm, often with eosinophilia. Acute myeloid transformation can occur.

Definitive Diagnostic Methods

Bone marrow biopsy
Genetic testing
Peripheral blood smear

Genetics Data

ETV6-JAK2
PCM1-JAK2 fusion
t(8;9)(p22;p24.1)
t(9;12)(p24.1;p13.2)
t(9;22)(p24.1;q11.2)

Immunophenotyping

None

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures

Transformations to

There are no known transformations

Transformations from

There are no known transformations

Corresponding ICD-9 Codes

238.79 Other lymphatic and hematopoietic tissues

Corresponding ICD-10 Codes

D47.7 Other specified neoplasms of uncertain or unknown behavior of lymphoid, hematopoietic, and related tissue

Corresponding ICD-10-CM Codes (U.S. only)

D47.Z9 Other specified neoplasms of uncertain behavior of lymphoid, hematopoietic and related tissue (effective October 01, 2015)

Signs and Symptoms

Easy bruising or bleeding
Fatigue
Fever
Frequent infections
Hepatosplenomegaly
Pain or a feeling of fullness below the rib
Pale skin
Petechiae
Shortness of breath
Weakness
Weight loss

Diagnostic Exams

Blood chemistry studies
Cytogenetic analysis
Immunocytochemistry
Peripheral blood smear

Progression and Transformation

Transformation to acute myeloid leukemia

Epidemiology and Mortality

Age range (12-75 years), with a median age of 47 years
Marked male predominance with male-to-female ratio of 27:5
Survival for chronic phase: ranges from several days to many years

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: Myeloid/lymphoid neoplasms with eosinophilia gene rearrangement
Pages: 78-79

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
Glossary