Many people with unicentric Castleman disease don't notice any signs or symptoms. The enlarged lymph node may be detected during a physical exam or an imaging test for some unrelated problem.
Some people with unicentric Castleman disease might experience signs and symptoms more common to multicentric Castleman disease, which may include:
- Fever
- Unintended weight loss
- Fatigue
- Night sweats
- Nausea
- Enlarged liver or spleen
The enlarged lymph nodes associated with multicentric Castleman disease are most commonly located in the neck, collarbone, underarm and groin areas.
CAUSES
It's not clear what causes Castleman disease. However, infection by a virus called human herpesvirus 8 (HHV-8) is associated with multicentric Castleman disease.
The HHV-8 virus has also been linked to the development of Kaposi's sarcoma, a cancerous tumor that can be a complication of HIV/AIDS. Studies have found that HHV-8 is present in nearly all HIV-positive people who have Castleman disease, and in about half of HIV-negative people with Castleman disease.
A rare disorder in which benign (not cancer) growths form in lymph node tissue. There are two main ways that Castleman disease occurs: localized (unicentric) and multicentric. Unicentric Castleman disease affects only one group of lymph nodes in one part of the body, usually in the chest or abdomen.
Treatment options for multicentric Castleman disease may include: Immunotherapy. The use of drugs such as siltuximab (Sylvant) or rituximab (Rituxan) can block the action of a protein that is produced in excess in people who have multicentric Castleman disease. Chemotherapy.
What is the prognosis (outlook) for people with Castleman disease? The outlook is very good for most people with unicentric Castleman disease (UCD) who have the affected lymph node removed. Surgery is typically considered curative. When treated, this condition does not usually affect life expectancy. The classic CT appearance of hyaline vascular Castleman disease is that of a solitary enlarged lymph node or localized nodal masses that demonstrate homogeneous intense enhancement after contrast material administration.
- D47.Z2 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
- The 2022 edition of ICD-10-CM D47.Z2 became effective on October 1, 2021.
- This is the American ICD-10-CM version of D47.Z2 - other international versions of ICD-10 D47.Z2 may differ.
Code Also- , if applicable, human herpesvirus 8 infection (B10.89)
The following code(s) above
D47.Z2 contain annotation back-references
that may be applicable to
D47.Z2:
- C00-D49 Neoplasms
- D37-D48 Neoplasms of uncertain behavior, polycythemia vera and myelodysplastic syndromes
- Angiofollicular lymph node hyperplasia
- Castleman's disease
ICD-10-CM D47.Z2 is grouped within Diagnostic Related Group(s) (MS-DRG v39.0):- 820 Lymphoma and leukemia with major o.r. Procedures with mcc
- 821 Lymphoma and leukemia with major o.r. Procedures with cc
- 822 Lymphoma and leukemia with major o.r. Procedures without cc/mcc
- 823 Lymphoma and non-acute leukemia with other procedures with mcc
- 824 Lymphoma and non-acute leukemia with other procedures with cc
- 825 Lymphoma and non-acute leukemia with other procedures without cc/mcc
- 840 Lymphoma and non-acute leukemia with mcc
- 841 Lymphoma and non-acute leukemia with cc
- 842 Lymphoma and non-acute leukemia without cc/mcc
Convert D47.Z2 to ICD-9-CM
Code History- 2017 (effective 10/1/2016): New code
- 2018 (effective 10/1/2017): No change
- 2019 (effective 10/1/2018): No change
- 2020 (effective 10/1/2019): No change
- 2021 (effective 10/1/2020): No change
- 2022 (effective 10/1/2021): No change
Diagnosis Index entries containing back-references to D47.Z2:
- Disease, diseased - see also Syndrome
- Castleman (unicentric) (multicentric) D47.Z2
- HHV-8-associated D47.Z2 - see also Herpesvirus, human, 8
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