History

1
History

• 37 year old admitted to WFUBMC for worsening
  dyspnea on 11/8/04.
• Had been having increasing shortness of breath
  for 2 weeks.
• Had a history of asthma, and was evaluated by
  pulmonary 3 days prior to admission.

2
History - 2

• CXR in the clinic had shown hilar fullness with
  bilateral lung opacities.
• He was also noted to have lichenified hyper
  pigmented lesions on his legs and back.
• A diagnosis of possible sarcoidosis was made and
  he had been placed on inhalers.

3
History - 3

• He had a mild cough with clear sputum.
• No chest pains, and a low grade fever.
• PMHx Asthma, anal fistulectomy 1991. Skin
  lesions for which dermatology had given him a
  steroid cream.
• MEDS Albuterol MDI, Flonase
• ALL NKDA
• SHx Single, works as a substance abuse
  counselor. No ETOH, Tobacco or drug use.

4
Physical Exam

• Vitals 132/68, P-115, RR-24, T-98.6
• HEENT Oral thrush, otherwise unremarkable.
• NECK Supple. Dark hyper pigmented lesions on his
  back.
• LUNGS Bilateral reduced AE, with occasional
  crackles and ronchi.
• ABD Soft non tender, BS pos
• EXT Several areas of thickened lichenified dark
  lesions on both legs

5
CXR 11/8/04
6
CXR Report

• CONCLUSION
• 1.  Diffuse patchy air-space opacities and
  interstitial prominence, unclear exact etiology,
  may represent edema, or an infectious etiology.
   Clinical correlation is recommended.
• 2.  Hilar and right paratracheal fullness
  worrisome for adenopathy.

7
CT Scan
8
CT Report

• CONCLUSION
• 1. Parenchymal findings with diffuse airspace
  opacities would be consistent, though not
  diagnostic for, provided clinical diagnosis of
  sarcoidosis. Additional considerations include
  organizing pneumonia, desquamative interstitial
  pneumonitis (DIP) and nonspecific interstitial
  pneumonitis (NSIP).
• 2. 9 mm sized right lower lobe nodule which may
  be unrelated to the remaining parenchymal
  findings is present. A 3-6 month chest CT is
  recommended to evaluate the stability of this.

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LABS

• 13
• 5.0 459 ACE Level 29 (normal)
• 38
• 49 segs, 32L (ALC 1600)
• 144 102 17
• 120
• 4.2 25 1.0

11
More Labs

• PNEUMOCYSTIS BY IMF POS
• SKIN, ABDOMEN, PUNCH BIOPSY
• Changes consistent with Kaposi Sarcoma
• HIV,RNA QUANTITATION 350000
• HIV, RNA LOG 5.5
• T-HELPER LYMPHS 0.02 X1000

12
Diagnosis?

• Kaposis sarcoma with advanced HIV and PCP
  pneumonia.

13
Kaposis Sarcoma

• In 1872, Moriz Kaposi 1st described
  idiopathsches multiples pigmentarskom der haut.
• Hungarian physician, was a dermatology faculty at
  the University of Vienna.
• Described 5 patients who all had a universally
  fatal outcome.

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Kaposis Sarcoma - 2

• Classic Kaposis is however typically an indolent
  neoplasm.
• Much more aggressive course in AIDS.
• Amongst AIDS patients, more common in MSM.
• In 1994, HHV-8 was described as an etiological
  factor in KS.

15
HHV-8

• Also known as KS associated herpes virus.
• HHV-8 is necessary, but not sufficient for
  development of all types of KS.
• HHV-8 has low prevalence in US and UK,
  intermediate rate in Italy, and high in Uganda.
• It is highly prevalent in MSM (25).
• Thought to spread through saliva.

16
Epidemiology

• Four groups
• Older men of Mediterranean and Jewish lineage.
• Africans in Uganda, Congo, Burundi and Rwanda.
• Iatrogenically immunosuppressed.
• MSM
• KS is the most common neoplasm in AIDS.
• Most iatrogenic KS patients are HHV-8 prior to
  immunosppression.
• KS more common in cyclosporin treated patients.

17
Clinical Features

• Multiple vascular nodules in skin and other
  organs.
• Can vary from localized Cutaneous dx to extensive
  involvement.
• Morphology varies
• Patch, plaque, nodular, lymphadenopathic,
  exophytic, infilterative, telangiectatic,
  keloidal and cavernous.

18
Clinical Features - 2

• Usually first noticed as plaques or nodules.
• Cutaneous lesions may rarely be infilterative or
  exophytic.
• Visceral KS is most evident in the GI tract and
  lymph nodes.
• Can however involve any organ.

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Kaposis Sarcoma
20
Endemic Kaposis Sarcoma
21
Ecchymotic KS
22
Oral KS
23
AIDS related KS
24
DDx

• Pyogenic granuloma
• Tufted angioma
• Melanocytic nevi.
• Cavernous hemangioma.
• Bacillary angiomatosis.
• AV maformations.
• Myofibromatoma.

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Pathology

• Characterized by proliferation of abnormal
  vascular structures.
• 3 types
• Spindle cell variant.
• Anaplastic form.
• Mixed cell form.
• Mixed cell form is the most common in AIDS
  patients.
• Large endothelial cells with proliferation of
  spindle cells and extravasation of erythrocytes.

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Treatment

• FDA approved therapies include
• Liposomal daunorubicin.
• Liposomal doxorubicin.
• Paclitaxel.
• Interferon alpha.
• Alitretinoin 0.1 for topical application.
• KS in AIDS has a poor 3 year survival rate.
• However Patients on HAART have a better survival
  rate.
• Previous treatment for herpes also improves
  survival.