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Hyaline vascular Castleman disease relapsing as T cell rich B cell lymphoma with paraneoplastic pemphigus
  1. Meeta Singh,
  2. Vijay Saroha,
  3. Parul Gupta,
  4. Praveen Gupta,
  5. Nita Khurana,
  6. Tejinder Singh
  1. Department of Pathology and Medicine, Maulana Azad Medical College, New Delhi, India
  1. Correspondence to Dr Meeta Singh, Room No 62, Maulana Azad Medical College, New Delhi-110002, India; meetamamc{at}

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A 32-year-old woman presented with mild fever and solitary discrete cervical lymph node (5 cm) for 2 years. The excision biopsy showed preserved lymph-node architecture with prominent onion skinning (figure 1) and hyalinised blood vessels in the germinal centres (figure 1, inset). The patient was investigated thoroughly for lymph nodes else where, but no lymph nodes could be detected. The overall picture was suggestive of localised Castleman disease (LCD), hyaline vascular type. The HIV, HHV-8 and Epstein Barr Virus serology was negative.

Figure 1

Hyaline vascular Castleman disease with prominent onion skinning and hyalinised blood vessels (H& E staining, 100×; inset: 400×).

The patient returned 5 years later with abdominal distension, weight loss, cervical and inguinal lymphadenopathy, hepatoslenomegaly and vescicobullous skin rashes for 3 months. CT and MRI revealed multiple cervical, retroperitoneal and mediastinal lymph nodes (figure 2).

Figure 2

CT abdomen and MRI neck (inset) showing enlarged lymph nodes.

Lymph-node biopsy showed complete effacement of architecture and infiltration of subcapsular sinus by large and small lymphoid cells (figure 3). On immunohistocemistry, the larger cells were CD 19 positive, while the smaller ones were positive for CD 45 RO (figure 3, inset). CD 30 and CD 15 were negative. Thus, a diagnosis of T cell rich B cell lymphoma was made. The skin and mucosal biopsy showed the presence of intraepidermal bullous lesion with granular deposition of C3 at the basement membrane and IgG at the intercellular space in the epidermis on immunofluorescence examination suggestive of pemphigus (figure 4).

Figure 3

T cell rich B cell non-Hodgkin's lymphoma (H&E staining, 400×; inset: immunohistocemistry with Avidin–Biotin method and DAB as chromogen, 400×) T cell: CD 45 RO, B cell: CD 19.

Figure 4

Paraneoplastic pemphigus (H&E staining, 400×).

Thus, a final diagnosis of a LCD relapsing as disseminated T cell rich B cell lymphoma with paraneoplastic pemphigus was made.

The patient was started on CHOP regimen and is well 2 years postchemotherapy.

CD is a heterogeneous disease that displays varied clinical manifestations. Malignancy in association with Castleman disease (CD) has been described previously.1 2 Development of Hodgkin's disease is well known and is more often associated with PC type CD.3 4

Non-Hodgkin's lymphomas (NHL) are more often associated with multicentric Castleman disease (MCD) than LCD with an incidence as high as 18% of MCD cases.1 The diagnosis of MCD-NHL can be concurrent or occurs within 2 years of the initial detection, while that of LCD-NHL is stated to be 45.6 months (0–156).5 B-NHL is the predominant type in both forms.

The present case adds a new case to the literature and supports the existing data on LCD regarding the age of presentation, which was 32 years in the present case, and its localisation to neck region. HIV and HHV 8 serology was negative in our case, and histopathology showed features of the hyaline vascular subtype. Its development into B cell NHL also corroborates well with the literature. However, the present case is unique in that T cell rich B cell immunophenotype has not been reported previously in a setting of LCD. This case also showed an interesting finding of coexisting Para neoplastic Pemphigus which is known to occur with NHL as well as Castleman disease.

NHL associated with CD is usually high grade and presents at stage III or IV at diagnosis, as in this case. Therefore, it has an aggressive clinical course and poor prognosis.5 Combination chemotherapy such as cyclophosphamide, vincristine and prednisone regimens, or Cyclophosphamide, Hydroxydaunorubicin, Oncovin and Prednisone regimens for treatment of Non Hodgkin's Lymphoma regimens, has an established role in CD-NHL treatment.

Thus, CD is a rare condition with an obscure pathogenesis. With preneoplastic potential in the hyaline vascular type, simple excision of affected lymph node may not be sufficient in LCD.

Take-home message

  • Owing to the preneoplastic potential in the hyaline vascular type, simple excision of the affected lymph node may not be sufficient in localised Castleman disease.



  • Competing interests None.

  • Patient consent Obtained.

  • Ethics approval Ethics approval was provided by the Maulana Azad Medical College Ethics Committee.

  • Provenance and peer review Not commissioned; externally peer reviewed.