Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:2.7.10.2 (focal adhesion kinase)
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Multicentric Castleman's disease (MCD) is a rare lymphoproliferative disorder. The precise incidence is unknown, although it is more common in HIV-positive than HIV-negative individuals. The pathological features of MCD strongly suggest a chronic antigen stimulation response, and human herpes virus 8 (HHV8) has been found to be universal in cases of HIV-related MCD. The presentation is non-specific with a wide differential diagnosis, which often results in a significant delay in its diagnosis. Diagnosis is made on the clinical presentation of a lymphoproliferative disorder, with evidence of multisystem involvement with classical histopathology on lymph node biopsy. Although no standard of care has been established for its treatment, symptomatic recurrences are often treated with corticosteroids and chemotherapy. The contribution of highly active antiretroviral treatment to the treatment of MCD remains debated. Novel treatments targeted at HHV8 show promising results, although evidence is currently limited to case reports. Randomized control trials assessing whether 'prophylactic' treatment with ganciclovir may prevent flares as currently used against cytomegalovirus disease in transplant patients are proposed. The prognosis of MCD in HIV-positive patients remains generally poor with a median survival of 48 months from diagnosis, and a 15-fold increased risk of non-Hodgkin's lymphoma.
Int J STD AIDS 2006 Jan
PMID:Multicentric Castleman's disease in HIV infection. 1651 1

Multicentric Castleman's disease (MCD) was originally described in non-HIV patients. It is a rare lymphoproliferative disorder, which is more commonly seen in HIV-positive patients and is associated with human herpes virus-8 (HHV-8). We describe a patient with advanced HIV who responded well to conventional highly active antiretroviral treatment. She was diagnosed with MCD soon after her diagnosis of HIV. She presented with multiple flares of her MCD. The case illustrates the difficulty of differentiating between episodes of septicaemia and a flare of MCD. The patient was treated with various chemotherapy regimens, which included several cycles of liposomal doxyrubicin and etoposide. There is currently no consensus on the treatment of MCD and various therapies are described in the literature, which include chemotherapy. Chemotherapy must be chosen with the immunosuppressive effects of the treatment being considered with caution. Both doxyrubicin and etoposide are well tolerated and successfully controlled the symptoms of MCD in our patient.
Int J STD AIDS 2006 Jan
PMID:Multicentric Castleman's disease in a patient with HIV. 1640 83

The objective of this study is to audit the implementation and knowledge of the British HIV Association (BHIVA) UK National guidelines for HIV testing (2008) in key conditions at Basildon & Thurrock University Hospital. Basildon Hospital is a district general hospital, serving over 400,000 patients in south-west Essex. A total of 348 patients were assessed through electronic to pathology data and patients' notes to investigate if they had been tested for HIV when diagnosed with the following conditions: tuberculosis (TB), hepatitis B and C, cervical intraepithelial neoplasia grade II/III, lymphoma, anal cancer, seminoma or Castleman's disease. The physicians involved were questioned as to their knowledge of the HIV testing guidelines. Of the 348 patients who were identified as having the above mentioned conditions, only 13.8% of those with any of the key conditions had received an HIV test. Only one non-HIV physician was aware of the guidelines. Knowledge of the 2008 BHIVA HIV testing guidelines is scanty among non-HIV-trained physicians. Health-care professionals in the field, irrespective of their role, should work harder to disseminate information and reduce prejudice that decreases testing of at-risk individuals.
Int J STD AIDS 2011 Feb
PMID:Assessment of the implementation and knowledge of the UK National Guidelines for HIV Testing (2008) in key conditions at a UK district general hospital. 2142 32

We present a case of primary effusion lymphoma (PEL) occurring simultaneously with Castleman's disease in the same patient. Castleman's disease is distinct from PEL, although both are associated with HHV-8. Other cases have debated whether the coexistence of PEL and Castleman's disease is a recurrence of an original PEL tumour in an extracavitary site, or a secondary HHV-8-associated lymphoma distinct from primary PEL. Our case, along with those described previously, show that co-occurrence of PEL and Castleman's disease is possible and plausible. PEL needs to be included in the differential diagnosis in any HIV-positive patient who presents with a pleural effusion, and diagnosis requires only a simple thoracentesis and appropriate immunohistochemistry.
Int J STD AIDS 2014 May
PMID:Castleman's disease and primary effusion lymphoma in a HIV-positive patient. 2428 26

Multicentric Castleman's disease (MCD), a polyclonal lymphoproliferative disorder of unknown aetiology, is a well-recognised complication of HIV disease. We present a case of MCD in an HIV-positive patient that is unusual on two counts: our patient's MCD first presented in the context of an immune restoration inflammatory syndrome (IRIS), following the initiation of highly active antiretroviral therapy (HAART). In addition, her MCD was associated with the unusual complication of acquired angioedema (AAE), which resolved following treatment of the MCD. While AAE is frequently found to have an underlying diagnosis of a lymphoproliferative disease, this is the first reported case linking AAE to MCD.
Int J STD AIDS 2014 Jun
PMID:A case of multicentric Castleman's disease in HIV infection with the rare complication of acquired angioedema. 2435 25