Castleman disease, angiofollicular lymph node hyperplasia, lymphoid hamartoma. TAFRO syndrome 4.33/5 (6)

Last updated: 25 / 07 / 22
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Definition

Castlemans Disease, Affiliation: Department of Radiology, Cork University Hospital, Cork, Ireland. Open

Castleman's disease is a benign, rare disease that causes swollen lymph nodes in different locations, most commonly in the chest (mediastinum), but can also be seen on the neck. The condition was described by Benjamin Castleman in 1956. Unifocal disease attacks a single lymph node, whereas multifocal disease attacks multiple areas. The multifocal type is divided into HIV-related and idiopathic type (without known cause). One form of the idiopathic multifocal type is the TAFRO syndrome (see below). Another multifocal form is similar POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disease and skin (skin), but distinguished from "primary" POEMS (Dispensary A, 2019). Castleman's disease is usually managed in hematology or infectious medicine.

Symptoms

Uni-centric is the most common type. Just en lymph node is attacked. It starts with a large, well-defined lymph node without particular pain. Rare changes in blood tests.

Multi-centric type is characterized by several enlarged lymph nodes, of which approx. 50% are associated with Kaposi's sarcoma herpesvirus (HHV-8), Please note! HIV infection. General symptoms include fever, anemia, nausea, weight loss.

Castleman disease
Castleman's disease with filling in the kidney. Saed-Abdul-Rahman J. CC BY NC 3.0

Examinations

Medical history in the unicentric type usually reveals no signs of a triggering cause and the symptoms (see above) are often minor. In the multicentric type, fever, nausea and exhaustion may be present.

Clinical can be felt by swollen lymph nodes can be felt on the neck, in the armpits and groin. The abdominal area is assessed for possible enlarged liver and spleen.

Blood tests may show elevated inflammation tests (CRP, SR), low number of red blood cells/hemoglobin (anaemia), low platelets (thrombocytopenia). In the multicentric type, liver and kidney function tests may be affected. Electrophoresis with immunofixation and HIV test can also be considered.

Imaging with CT examinations of the lung area (thorax), stomach area (abdomen) and pelvis, alternatively used PET / CT which is useful for defining spread (uni- or multifocal). Tissue with the highest FDG uptake should be selected for tissue sampling (reference: Koa B, 2021).

Tissue sample (biopsy) shows capsular fibrosis and broad bands of fibrosis passing through the lymph node. Increased number of lymphoid follicles scattered over the cortex and medulla, often with several germinal centers within the mantle zone. The mantle zones have an onion peel pattern (reference: Dispenzeri A, 2020).

TAFRO syndrome

TAFRO syndrome shown on PET/CT. Nagano M, Matsumoto J – Surg Case Rep (2016). CC BY 4.0

Definition. TAFRO syndrome is a variant of multicentric Castleman's disease and has been identified in Japan. It is described among middle-aged and elderly people, less often among young people. TAFRO can appear simultaneously with other diseases, incl autoimmune rheumatic diseases, infections and cancer. (Literature: Kawabata H, 2013, Konishi Y, 2015).

The syndrome consists of (Srkalovic G, 2017):

  • Thrombocytopenia (low platelet count)
  • Anasarca (fluid in the body)
  • Reticulin fibrosis in bone marrow (biopsy)
  • Renal function is reduced
  • Organomegaly (large organs)

Similar conditions, differential diagnoses

Treatment

Uni-centric type: Surgical removal is 90% successful without recurrence. Multicentric type: Antiviral treatment in HIV. Immunosuppressive drugs. Siltuximab (IL-6 inhibitor) showing promising results (Lancet 2014).

Literature

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