Marginal zone b cell lymphoma icd 10

Marginal zone b cell lymphoma icd 10

Name

ICD-O-3 Morphology

Effective 2001 and later

Reportable

for cases diagnosed 2001 and later

Primary site must be spleen (C422)

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Abstractor Notes

Diagnostic Confirmation

This histology can be determined by positive histology (including peripheral blood) with or without genetics and/or immunophenotyping. Review the Definitive Diagnostic Methods, Immunophenotyping and Genetics Data sections below, and the instructions in the Hematopoietic Manual for further guidance on assigning Diagnostic confirmation.

Alternate Names

Definition

Definitive Diagnostic Methods

Genetic testing

Histologic confirmation

Immunophenotyping

Genetics Data

Dysregulation of CDK6 gene at 7q21

Immunoglobulin heavy and light chain rearrangement

Immunophenotyping

Annexin A1 neg

CD5-

CD10-

CD20+

CD23-

CD43-

CD79a+

CD103-

Cyclin D1 absent

Ki-67 staining shows distinctive targetoid pattern

LEF1 absent

Surface IgD

Surface IgM

Treatments

Chemotherapy

Immunotherapy

Surgery

Transformations to

Transformations from

None

Same Primaries

Corresponding ICD-9 Codes

200.3 Marginal zone lymphoma

Corresponding ICD-10 Codes

C83.0 Non-Hodgkin lymphoma small cell (diffuse)

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

C83.0 Small cell B-cell lymphoma (effective October 01, 2015)

Signs and Symptoms

Anemia

Drenching night sweats

Fatigue

Fever (for no known reason)

Pain in the chest, abdomen, or bones (for no known reason)

Skin rashes or itchy skin

Splenomegaly

Weight loss (for no known reason)

Diagnostic Exams

Flow cytometry

Immunohistochemistry

Immunophenotyping

Splenectomy

Progression and Transformation

Epidemiology and Mortality

Age: mostly 50 years and older

Incidence: 2% of lymphoid neoplasms

Sex: no male or female predominance

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: Mature B-cell neoplasms
Pages: 223-225

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

National Cancer Institute
Section: General Information About Adult Non-Hodgkin Lymphoma (NHL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq

Marginal zone b cell lymphoma icd 10

C88.4 - Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue [MALT-lymphoma] is a topic covered in the ICD-10-CM.

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Code

C88.4 - Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue [MALT-lymphoma]
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Code

C88.4 - Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue [MALT-lymphoma]
[Billable]

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Disease definition

A rare, indolent primary cutaneous B-cell lymphoma characterized by multifocal, red to violaceous papules, plaques or nodules localized predominantly on the trunk and extremities. Histologically, these are dermis infiltrates consisting of small, marginal zone B cells, lymphoplasmacytic cells, and plasma cells. Marginal zone B cells express CD20, CD79a and Bcl-2, and are negative for CD5, CD10 and Bcl-6. Plasma cells are typically located at the periphery, and express CD138, CD79a, and monotypic light chains.

ORPHA:178536

Classification level: Disorder

  • Synonym(s):
    • PCMZL
  • Prevalence: -
  • Inheritance: -
  • Age of onset: -
  • ICD-10: C83.0
  • ICD-11: 2A85.2
  • OMIM: -
  • UMLS: C1275321
  • MeSH: D018442
  • GARD: -
  • MedDRA: -

The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.

What is marginal zone B

Marginal zone lymphoma (MZL) is a group of indolent (slow growing) NHL B-cell lymphomas, which account for approximately eight percent of all NHL cases. The average age at diagnosis is 60 years, and it is slightly more common in women than in men.

Is marginal zone lymphoma Hodgkin's or non Hodgkins?

Marginal zone lymphoma (MZL) is the second most common indolent non-Hodgkin's lymphoma (iNHL). There are three types of marginal zone lymphomas: the extranodal MZL (EMZL) of mucosa-associated lymphoid tissue (MALT or gastric GALT), the splenic MZL, and the nodal MZL.

Where is marginal zone lymphoma?

Splenic marginal zone B-cell lymphoma is a more rare lymphoma found in blood, bone marrow and the spleen. As with other types of MZL, it tends to grow and spread slowly, and treatment may be safely delayed unless symptoms or other problems arise.

Is marginal zone lymphoma the same as MALT lymphoma?

MALT lymphoma is a slow-growing (low-grade) non-Hodgkin lymphoma that develops from B cells. It is a type of marginal zone lymphoma: a lymphoma that develops in a particular region at the edge of lymphoid tissues (collections of lymphocytes) called the marginal zone.