Name
ICD-O-3 Morphology
Effective 2001 and later
Reportable
for cases diagnosed 2001 and later
Primary site must be spleen (C422)
Help me code for diagnosis year :
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: //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: //www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
C88.4 - Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue [MALT-lymphoma] is a topic covered in the ICD-10-CM. To view the entire topic, please log
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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: -
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