Name

Lymphocyte-depleted classic Hodgkin lymphoma

ICD-O-1 Morphology

9653/3: Hodgkin lymphoma, lymphocytic depletion, NOS
Effective 1978 - 1991

ICD-O-2 Morphology

9653/3: Hodgkin lymphoma, lymphocytic depletion, NOS
Effective 1992 - 2000

ICD-O-3 Morphology

9653/3: Hodgkin lymphoma, lymphocytic depletion, NOS
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

C770-C779
See Module 7. Lymph nodes (C770-C779) are the usual primary sites; however, involvement in other sites is possible. If you have confirmation that the only involved site is something other than lymph nodes, then code to that primary site.

Abstractor Notes

Lymphocyte-depleted classical Hodgkin lymphoma (LDCHL) has a predilection for retroperitoneal lymph nodes, abdominal organs, and bone marrow. Compared to other subtypes of CHL, LDCHL presents at a more advanced stage (III-IV) and with B symptoms.

Prior to modern therapy the course of LDCHL was aggressive and it has remained so in some parts of Europe and developing countries.

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.

Grade

Not Applicable

Module Rule

None

Alternate Names

Classical Hodgkin lymphoma, lymphocyte depletion, NOS
Hodgkin lymphoma, lymphocyte depletion, NOS
LD-HL
LDCHL

Definition

Lymphocyte-depleted classic Hodgkin lymphoma (LDCHL) is a diffuse form of classic Hodgkin lymphoma (CHL) rich in Hodgkin/Reed-Sternberg (HRS) cells and/or depleted of non-neoplastic lymphocytes. Histiocytes are usually abundant.

Definitive Diagnostic Methods

Histologic confirmation
Immunophenotyping

Genetics Data

None

Immunophenotyping

CD30 coexpression
CD79a negative
EBV/LMP1 positive
OCT2 expression
PAX5 coexpression

Treatments

Chemotherapy
Hormone therapy
Radiation therapy

Corresponding ICD-9 Codes

201.7 Hodgkin's disease, lymphocytic depletion

Corresponding ICD-10 Codes

C81.3 Hodgkin lymphocytic depletion

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

C81.3 Lymphocyte depleted Hodgkin lymphoma (effective October 01, 2015)

Signs and Symptoms

Drenching night sweats
Fatigue
Fever (for no known reason)
Pain in the chest, abdomen, or bones (for no known reason)
Painless swelling in the lymph nodes
Retroperitoneal lymphadenopathy
Skin rash or itchy skin
Weight loss (for no known reason)

Diagnostic Exams

Blood chemistry studies
CT (CAT) scan
Erythrocyte sedimentation rate
Immunophenotyping
Lymph node biopsy
PET (positron emission tomography) scan

Progression and Transformation

None

Epidemiology and Mortality

Age: 30-37 years median age
Incidence: <1% of classical Hodgkin lymphomas
Sex: male predominance
Survival: good prognosis

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: Hodgkin lymphomas
Pages: 441-442

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 Hodgkin Lymphoma (HL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-hodgkin-treatment-pdq
Glossary