Castleman's disease

  • slide 27
  • Prof. Hirschel

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1/27 MCD Therapy

Splenectomy

2/27 Case study: HHV8 Primary Infection, slide #1

Transient angiolymphoid hyperplasia and kaposi's sarcoma after primary infection with human herpesvirus 8 in a patient with human immunodeficiency virus infection.

3/27 Case Study: HHV8 Primary Infection, slide #2

Patient AD 1952- HIV (CD4 200/mm3; viremia 875,000 cp/ml)- Salmonellosis- Persistent fever >5 weeks- Bicytopenia (Hb: 100; platelets: 50)- Enlarged mediastinal lymph nodes; enlarged liver; pleural effusion, ascites- No effect of multiple antibiotics including anti-TB and HAART (Kaletra-d4T-3TC).

4/27 Case study: HHV8 primary infection, slide #3

Day 15: Kaposi's sarcoma in inguinal lymph node. Vascular proliferation. Extravasated red blood cells.

5/27 HHV8 primary infection slide #4

Day 20: - Rapidly worsening dyspnea - BAL: 79% macrophage; 20% lymphocytes; 1% polynuclear cells. No infectious agents were visible on Gram and acid fast stains.

6/27 Case Study: HHV8 Primary Infection, slide #5

Samples from Day 23 - Quantitative PCR in the blood : 11,000 HHV-8 cp/100 000 cells - Quantitative PCR in the pleural fluid: 40,000 HHV-8 cp/100 000 cells - Inguinal biopsy: PCR analysis for DNA-HHV-8 (recognizing ORF-75): strongly positive. - Anti HHV-8 antibodies negative.

7/27 HHV8 primary infection, slide #6

Day 20: Transfer into intensive care unit.

8/27 Case study: HHV8 primary infection, slide #7

Day 22: increase in size of lymph nodes and large bilateral pleural effusions.

9/27 Case Study: HHV8 Primary Infection, slide #8

Patient AD, continued- Day 23: treatment with anti-CD20 antibodies- Day 34: died of multi organ failure- Post-mortem plasma sample: positive for anti-HHV8 antibodies.

10/27 Case 2, slide #1

Multicentric Castleman's Disease.- 50 yo homosexual- July 2002: fatigue, 4 kg weight loss; CD4 176/mm3 (16%); viremie 256'000; start of HAART. - August 2002: Rapid development of lesions suggestive of Kaposi's sarcoma.

11/27 Case 2, slide #2

- September 2002: Increase in extent of cutaneous KS and lymph node swelling- Leg edema- Bronchoscopy: KS- Fever 38-39°C- Pancytopenia, in particular thrombocytopenia- January 2003. HHV8 antibodies postive; PCR: 18000 copies per 105 lymphocytes.

12/27 Case 2, slide #3

Massive enlargement of spleen.

13/27 Case 2, slide #4

Splenectomy (Jan 13, 2003)- Spleen weight 1536 g- Positive in-situ hybridization for HHV8- No evidence of lymphoma- No evidence for Kaposi's sarcoma.

14/27 Case 2, slide #5

- Etoposide 200 mg weekly for 4 weeks, then 150 mg weekly for 4 months- Disappearance of fever, thrombocytopenia and KS skin lesions- Relapse in 10/2003, treated with etoposide and rituximab until 4/2004- HHV-8 negative in plasma after 2004- On HAART, rise in CD4 cells from 156 to 1500.

15/27 Case 3, slide #1

Massive lymph node swellings in a case of biopsy-proven Multicentric Castleman's Disease.

16/27 Case 4, slide #1

- 28 year old African woman with HIV infection and a CD4 count of 38 on Aug 1/2001. Start of treatment with Kaletra, d4T, 3TC - September/October 2001: Fever, anemia (Hb 6.6), neutropenia (ANC 800), albumine 16 g/L, proteinuria. - Sputum:

17/27 Case 4, slide #2

Lymph node biopsy, with typical "onion-peel" appearance of lymphoid granuloma (see below).

18/27 Case 4, slide #3

Stains for both types of light chains are positive, suggesting a polyclonal population. In-situ immuno-histochemistry for HHV8 was also positive (not shown).

19/27 Case 4, slide #4, Gastric biopsy

Dense lymphocytic infiltrate in the submucosa. The stain for kappa chains is negative, while the stain for lambda chains is strongly positive.

20/27 Case 4, slide #5

- Treatment for tuberculosis with rifabutin, isoniazid, ethambutol, and pyryzinamide - Continued HAART - Hemolytic anemia: prednisone - Nov 18, 2001: Re-admitted for fever, pancytopenia, abdominal pain , abnormal liver function tests - Nov 23: Splenectomy for intractable thrombocytopenia and hemolysis- Nov 28: Coma, absent pupillary reflexes- Nov 29: died.

21/27 MCD; case 4, slide #6

CT image one day before the patient's demise, showing hypodense mass in right hemisphere.

22/27 Case 4 slide #7

Autopsy results from brain (left), and stomach (top right) showing fungal mycelia, compatible with actinomyces or mucormycosis.

23/27 MCD; case 5, slide #1

Kaposi/Castleman/Body Cavity Lymphoma - 34 year-old patient with HIV/HCV/HBV infection, treated intermittently with efavirenz and combivir - CD4 count of 457 (19%), viral load 5000- Fever, weight loss, anemia - Rapidly developing cutaneous and lymphatic Kaposi's sarcoma- Enlargement of liver and spleen.

24/27 MCD; case 5, slide #2

- October 2005: hepato-spleno-megaly- Treatment with liposomal donorubicin (Caelyx ®) - Clinicallly in remission

25/27 MCD; case 5, slide #3

October 2006: Renewed fever and pre-cordial pain.

26/27 MCD; case 5, slide #4

Frame from 2-dimensional echocardiogram, showing pericardial effusion (echo-free space, arrows).

27/27 MCD; case 5, slide #5

Giemsa stain of pericardial fluid, with large plasmocytoid cells, harbouring prominent nucleoli. These cells were CD20 negative, but CD38 positive. Polynuclear cells are also seen (thin arrows). The patient developed fever, renal failure, hypotension, and died despite administration of CHOP, less than 1 month after the diagnosis was established.

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