Saturday, 1 March 2014

Kaposi sarcoma as an AIDS-defining illness

Kaposi sarcoma (KS) is the commonest tumour in patients infected with HIV. It is an AIDS-defining illness and is caused by a γ herpesvirus, the Kaposi sarcoma herpesvirus (KSHV), also called Human Herpesvirus-8 (HHV-8). The diagnosis is usually based on the characteristic appearance of cutaneous or mucosal lesions and should be confirmed histopathologically.  The beneficial effects of HAART on both the incidence and the outcomes of KS have been shown in several studies. Many case reports and small series have described the regression of KS with HAART alone. Initiation of HAART may precipitate a paradoxical worsening of symptoms, termed the immune reconstitution inflammatory syndrome (IRIS)*. Sudden progression of existing KS or development of new lesions may occur. Independent risk factors for IRIS-KS development include pre-existing clinical KS, a higher CD4 cell count at the time of KS diagnosis (in contrast to general IRIS predictors**, no association between IRIS-KS development and low pre-HAART CD4+ cell counts has been observed), the presence of tumour associated oedema, pre-HAART haematocrit value of less than 30%, pre-HAART detectable plasma KSHV DNA, pre-HAART plasma HIV-1 RNA viral load (hazard ratio 34.6 per log viral load increase) and the use of protease inhibitors and nonnucleosides together in a single regimen. The clinical management of IRIS-KS is usually with systemic chemotherapy.

*Gohar A. Immune restoration Klebsiella lymphadenitis. International J STD AIDS 2011; 22:536-7.

**The relationship between low CD4 count and the development of IRIS may depend on the specific pathogens involved. In the case of KSHV, CD4+ lymphocyte depletion has not been shown to increase replication.

Summary of the relevant current BHIVA recommendations:
• It is recommended that KS should be confirmed histopathologically.
• It is suggested that CT scans, bronchoscopy and endoscopy are not warranted in the absence of symptoms.
• It is recommended that HAART should be started in all patients diagnosed with KS.
• It is suggested that local radiotherapy or intralesional vinblastine for symptomatic or cosmetic improvement in early stage T0 KS.
• It is recommended that patients with T1 advanced stage KS, should receive chemotherapy along with HAART.
• It is recommended that liposomal anthracyclines (either DaunoXome 40 mg/m2 q14d or Caelyx 20 mg/m2 q21d) are first line chemotherapy for advanced KS.
• It is recommended that paclitaxel chemotherapy (100 mg/m2 q14d) for second-line treatment of anthracycline refractory

• All patients should be considered for clinical trial enrolment if eligible.
This page was last updated in November 2014

Main Works of Reference List (The first eight are my top favourites)

  • British National Formulary
  • British National Formulary for Children
  • Guidelines (BAD - BASHH - BHIVA - Uroweb)
  • Oxford Handbook of Genitourinary Medicine, HIV, and Sexual Health
  • Oxford Handbook of Medical Dermatology
  • Rook's Textbook of Dermatology
  • Simple Skin Surgery
  • Weedon's Skin Pathology
  • A Concise Atlas of Dermatopathology (P Mckee)
  • Andrews' Diseases of the Skin
  • Andrology (Nieschlag E FRCP, Behre M and Nieschlag S)
  • Bailey and Love's Short Practice of Surgery
  • Davidson's Essentials of Medicine
  • Davidson's Principles and Practice of Medicine
  • Fitzpatrick's Colour Atlas and Synopsis of Clinical Dermatology (Klaus Wolff FRCP and Richard Allen Johnson)
  • Fitzpatrick’s Dermatology in General Medicine
  • Ganong's Review of Medical Physiology
  • Gray's Anatomy
  • Hamilton Bailey's Demonstrations of Physical Signs in Clinical Surgery
  • Hutchison's Clinical Methods
  • Lever's Histopathology of the Skin
  • Lever's Histopathology of the Skin (Atlas and Synopsis)
  • Macleod's Clinical Examination
  • Martindale: The Complete Drug Reference
  • Oxford Handbook of Clinical Examination and Practical Skills
  • Oxford Textbook of Medicine
  • Practical Dermatopathology (R Rapini)
  • Sexually Transmitted Diseases (Holmes K et al)
  • Statistics in Clinical Practice (D Coggon FRCP)
  • Stockley's Drug Interactions
  • Treatment of Skin Disease: Comprehensive Therapeutic Strategies
  • Yen & Jaffe's Reproductive Endocrinology