Large B-cell lymphoma arising in HHV8-associated multicentric Castleman disease

Name
Large B-cell lymphoma arising in HHV8-associated multicentric Castleman disease
ICD-O-3 Morphology
9738/3
Effective 2010 and later
Reportable
for cases diagnosed 2010 and later
Primary Site(s)
See Abstractor Notes and Module 7

Help me code for dx year :

Grade
6 - B-cell
Module Rule
None
Alternate Names
HHV8 MCD
HHV8 PL
HHV8-positive plasmablastic lymphoma
Kaposi Sarcoma herpes virus (KSHV)-positive plasmablastic lymphoma
Definition
This disease is composed of HHV8-infected lymphoid cells resembling plasmablasts expressing IgM and arising in the setting of multicentric Castleman disease (MCD). It is usually associated with HIV.
Abstractor Notes
(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010 see code 9680/3.) The lymphoma characteristically involves lymph nodes and spleen but can disseminate to other viscera via the blood stream in which rarely it manifests as a leukemia. On a background of HHV8 CD, patients who are developing HHV8 PL present with profound immunodeficiency, enlarging lymph nodes and massive splenomegaly often accompanied by manifestations of Kaposi's Sarcoma.

The term "plasmablastic" is used for this lymphoma because the cells resemble plasma cells and have abundant immunoglobulin, but they lack IG somatic hypermutation. This lymphoma is considered to be the same primary as plasmablastic lymphoma (9735/3) but the two diagnosis can be distinguished because plasmablastic lymphoma frequently show class-switched and hypermutated IG genes. Primary effusion lymphoma (9678/3) may complicate HHV8 MCD but PEL neoplastic cells do no express immunoglobulin and are usually co-infected with EBV.

This is a very aggressive lymphoma with a median survival of a few months.

Note: Castleman disease by itself is not reportable.
Definitive Diagnostic Methods
Histologic confirmation
Immunophenotype
Genetics Data
IG genes are unmutated
Immunophenotyping
CD20+ or CD20-
CD27-
CD38+/-
CD79a-
CD138-
cIgA positive
cIgM negative
Epstein-Barr encoded RNA (EBER)-negative
Nuclear staining for interleukin-6
Nuclear staining for LANA-1
Strongly express cIgM with lambda light chain restriction
Treatments
Chemotherapy
Immunotherapy
Stem cell transplant
Surgery
Transformations to
None
Transformations from
None
Corresponding ICD-9 Codes
202.8 Other lymphoma
Corresponding ICD-10 Codes
C85.7 Other specified types of non-Hodgkin's lymphoma
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C85.8 Other specified types of non-Hodgkin lymphoma
Signs and Symptoms
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None