Thanks to Wendy for presenting a case of an elderly man with h/o remote renal transplant presenting with chronic progressive DOE, lower extremity edema, and upper and lower GI bleed, found to have AIDS-related GI kaposi sarcoma and associated protein-losing enteropathy!

Clinical Pearls:

• Keep a broad differential for patients on immunosuppression
• Incidence of KS is higher with CD4 counts <200 but it can be seen in CD4>500 as well.
• Prognosis is generally good with treatment.  Poorer prognosis is associated with visceral involvement (as opposed to cutaneous), multiple opportunistic infections, and CD4<200
• Mainstay of therapy is anti-retrovirals.  Chemotherapy can be used for ARV unresponsive disease, significant edema, extensive organ involvement, or IRIS.  Studies on chemo + ARV vs ARV alone showed no survival benefit with the former.
• Thanks to Dr. Szumowski for the clinical pearl on use of sirolimus in renal transplant recipients with KS (article here).

Differential for odynophagia:

• Infectious
  • HSV
  • CMV
  • Fungal
    • Candida ⇒ risk factors include dentures, immunosuppression (AIDS, chemo), radiation to head and neck, recent antibiotics
    • Others: crypto, histo, blasto, aspergillus
  • Mycobacteria
• Medication-induced
• Less common
  • Reflux esophagitis
  • Crohn’s

Kaposi Sarcoma:

• Vascular tumor associated with HHV-8
• Four different epidemiologic forms:
  1. AIDS-related: most common type in US
     • Higher incidence with CD4 <200 but can be seen with CD4 >500 as well.
  2. Endemic/African
  3. Organ transplant-associated (higher incidence after solid organ transplant)
  4. Classic (indolent cutaneous proliferative disease in older men of Mediterranean or Jewish descent)

KS in the GI tract:

• Can occur in the absence of cutaneous disease
• Symptoms range from asymptomatic to weight loss, abdominal pain, n/v, UGIB/LGIB, malabsorption, diarrhea
• Inflammatory cytokine syndrome:
  • Systemic inflammation in AIDS-related KS
  • Symptoms:
    • Fever
    • Edema
    • Neuropathy
    • GI/respiratory symptoms
    • Hypoalbuminemia (can occur in the absence of the who syndrome)
      • Secondary to protein losing enteropathy (check stool clearance of alpha-1 antitrypsin)
    • Thrombocytopenia
    • Splenomegaly

• Extent of tumor (T): limited to skin with minimal oral cavity involvement is good.  Visceral involvement has poor prognosis.
• Immune status (I): Degree of immunosuppression from HIV. CD4 <200 has worse prognosis
• Severity of systemic illness (S): poor prognosis a/w h/o OI, thrush, B symptoms, etc.
• Endoscopy and bronchoscopy are only done if initial stool test and CXR are abnormal

• Goal: palliation, prevention of disease progression, and shrinkage of tumor to alleviate edema, organ compromise, and psychological distress
• Systemic
  • Treatment with potent ART
    • IRIS can occur within 3-6 weeks of initiation
  • Chemo: for patients with advanced KS and rapid progression
    • Indications
      • >25 lesions
      • Unresponsive to local treatment or ART alone
      • Extensive edema
      • Symptomatic visceral involvement
      • IRIS
    • Agents:
      • Pegylated liposomal doxorubicin or daunorubicin
      • Paclitaxel, bleo, vinblastine, vincristine, etoposide
  • Chemo + ART or ART alone? While response rates are higher with the former, no survival benefit
• Local symptomatic therapy
  • Intralesional chemo (vinblastine)
  • Radiation therapy
  • Topical alitretinoin