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ID Case Conference February 13, 2008 Carlos M. Perez, MD, FACP Associate Professor of Medicine Pontificia Universidad Catolica de Chile
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The Case HPI: ♂ 22 yo, HIV (+) diagnosed in May 2007 when he had elevated LFTs and positive CMV antigenemia (“Acute CMV infection with Hepatitis”). CD4 559 cells/mm3. HIV viral load 57,800 copies/mL. (-) RPR. (-) IgG Toxoplasma. (+) Anti core Ab Hepatitis B. (-) HBsAg. (-) Hepatitis C. CD4: 352 (June) 261 (July) 224 (August) HAART started in September: TDF+3TC+EFV
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The Case HPI: Two weeks after initiation of HAART developed a generalized maculopapular erythematous rash, fever, nasal congestion and sore throat. Symptoms did not improve with antihistaminics and Ibuprofen and the patient was admitted for further work up.
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PMH & PSH: “Viral meningitis” October 2006 Medications: TDF+3TC+EFV Allergies: Ketoprofen SH: MSM. Student. Tobacco ½ pack a day. Occasional ETOH. One sexual partner. No pets. No recent travel. ROS: As described
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Physical exam P: 100 x min BP:110/70 T: 39 °C, RR: 22 x min. Normal saturation. Skin: Generalized erythematous maculo papular rash. No mucosal involvement. Generalized lymphadenopathy (cervical, axillary, inguinal): Variable sizes, some tenderness. HEENT: Nasal congestion. Congestive Pharynx. No exudates. Lungs and Heart wnl Abdomen: Hepatosplenomegaly 3- 4 cm below costal margin. Extremities and Neurological examination wnl
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Discussion
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Laboratory
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7,9 Calcium 6,5 Proteins 3,0 Albumin 15 CRP 2,6 Phosphorus 122 Glucose 133 LDH 98 Alc P 20 ALT 0,52 Bili T 12 BUN 0,66 Creatinine Oct 20, 2007 Chemistry - T. Gondii IgM RSV Resp Viral Panel - Bartonella sp - IgM PVB19 +/- Ig G/IgM EBV - Ig M CMV/PP65 - Blood cultures Oct 22, 2007 Serologies
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Imaging CT Neck, Chest, Abdomen and Pelvis: Multiple lymphadenopathy. Moderate hepatosplenomegaly. Mild bilateral pleural effusion and Ascites.
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Imaging
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Discussion
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Lymph node Flow cytometry
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Lymph node Pathology (1) Angiofollicullar hyperplasia with hyaline vessels penetrating follicles. Increase lymphocytes in mantle zone with an “onion-skin” arrangement around the germinal centers.
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Lymph node Pathology (2) Spindle-shaped cells and neovascularization with aberrant proliferation of small vessels. Extravasations of erythrocytes and hemosiderin deposits.
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Lymph node Pathology (3) Nuclear immunoreactivity to HHV-8/KS latency nuclear antigen
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Conclusion: Multicentric Castleman’s Disease and Kaposi Sarcoma in an HIV- infected patient
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Clinical course HAART was maintained and patient's symptoms slowly improved without specific treatment. Conclusion: Multicentric Castleman’s Disease and Kaposi Sarcoma as Immune Reconstitution Syndrome
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Castleman´s disease Castleman's disease (CD, angiofollicular lymph node hyperplasia), is a lymphoproliferative disorder described in 1956 by Benjamin Castleman, who identified a series of patients with solitary hyperplastic mediastinal lymph nodes with small germinal centers resembling Hassall's corpuscles of the thymus. Two clinical foms: Unicentric and Multicentric (MCD). It is associated with HHV-8 (universally found in HIV+ MCD and 40-50% HIV- MCD).
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Castleman´s disease There is an immunoblastic hyperplasia and plasma cell formation. The mantle zone lymphocytes in CD are polyclonal IgM or IgD- expressing cells. The plasma cells in the interfollicular areas are generally also polyclonal, although localized clonal expansions are sometimes seen. Associated with other malignacies: KS (70%), NHL (15-20%), HL, POEMS Syndrome (polineuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes)
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Castleman´s disease Pathogenesis: It has been hypothesized that HHV-8 could be the triggering antigen for the induction of a pathological process via abnormal IL-6 production (viral and human IL-6). Clinical features: Median age 52-65 yo. 65% male. Fever (10 %), night sweats, weight loss, fatigue, dyspnea, peripheral lymphadenopathy (100%), hepatosplenomegaly (50%). Laboratory abnormalities include nearly universal anemia, hypoalbuminemia, hypergammaglobulinemia, and elevated sedimentation rate.
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Castleman´s disease Clinical course: Most patients with multicentric CD die of fulminant infection, progressive Castleman's disease or related malignancies. Treatment: Steroids, Chemotherapy, Antiviral agents (Cidofovir, Ganciclovir, Foscarnet), Inhibition of IL-6 ( Tocilizumab and Altlizumab), Thalidomide, Rituximab + HAART (median survival 48 months versus 14 months without HAART)
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Search PubMed Multicentric Castleman’s Disease and Kaposi Sarcoma in an HIV-infected patient –Case ReportsCase Reports –ReviewReview –Differential DiagnosisDifferential Diagnosis –Drug TherapyDrug Therapy Note: In order to see PubMed results, use View Slide Show, or hit F5
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