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Clinical Pathological Case Conference - Answer

Clinical Pathological Case Conference - Answer. Kristin Remus, D.O. Chief Resident NYU School of Medicine, Internal Medicine August 8, 2008. Radiology. Review of Radiology showed the following Normal Chest x-ray Lung nodule on Chest CT Normal Abdominal CT.

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Clinical Pathological Case Conference - Answer

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  1. Clinical Pathological Case Conference - Answer Kristin Remus, D.O. Chief Resident NYU School of Medicine, Internal Medicine August 8, 2008

  2. Radiology • Review of Radiology showed the following • Normal Chest x-ray • Lung nodule on Chest CT • Normal Abdominal CT

  3. A diagnostic test was performed: Endoscopy and Colonoscopy with biopsies

  4. Further Studies • Stool contained Strongyloides Stercoralis larva • Endoscopic studies did not show stigmata of recent bleeding • Lab tests • HIV negative • Lymph node biopsy was not performed • The patient had been offered screening colonoscopy 1 year prior and declined. • Biopsies negative for H. pylori

  5. Additional Lab Results Purkinje Cell Ab - negative Hu immunoreactivity – negative Anti-ganglioside IgM <1:800 Anti-ganglioside IgG <1:100 Iron ug/dL 70 (42-146) TIBC ug/dL 189 (250-450) Ferritin ng/mL 186.7 (22-322) Retic % 3.77 (0.5-1.55) Retic Index 2% PSA ng/mL 0.44 (0-4) CEA ng/mL <0.5 (<=5) CA-125 U/mL 14.2 (<=35) AFP ng/mL 1.5 (0-10) Serum ACE U/L 19 (9-67) Serum immunofixation – faint bands in IgG, IgM, and Kappa are present against a dense, polyclonal background.

  6. Strongyloides Stercoralis • Tropical Asia, Africa, Latin America, Southern US, Eastern Europe • May persist asymptomatically in host for up to 65 years • Risk factors for clinical manifestation • Chronic disease – Diabetes, Kidney Disease, Alcoholism • Immunosuppression • Hematologic malignancies • Malnutrition • HTLV-1 infection • Diagnosis • Parasite found in feces, sputum, duodenal aspiration, CSF, tissue biopsy

  7. Strongyloides Life Cycle parthenogenesis FECES SOIL infective larvae

  8. Strongyloides Stercoralis • Clinical Presentation • Skin • larva currens • GI tract • Cramps, diarrhea • Malabsorption • Rarely massive hemorrhage • Immunosuppressed • Fever • Lungs • larvae in sputum • Many fatalities reported Cutaneous larva currens, “racing larva”

  9. Stronglyoides Infection • Immunosuppresion • Steroids may mimic endogenous parasitic-derived regulatory hormone • More eggs produced in the presence of exogenous steroids • Hyperinfection • Disseminated infection • Treatment • oral Ivermectin 200 ug/kg daily x 2 days, Albendazole as alternative • Prevention • CDC recommends oral Ivermectin 200 ug/kg daily x 2 days for prevention in immunosuppressed • In a least one study, Thiabendazole was no more effective than placebo

  10. Chronic Acquired Demyelinating Polyneuropathy (CADP) • A group of peripheral nerve disorders • Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is a type of CADP • Peak incidence 40 to 60 years, male predominance • Pathophysiology unclear

  11. CIDP Diagnostic Features • Symmetric proximal and distal muscle weakness • +/- sensory loss • Loss of deep tendon reflexes • Progressive or relapsing • Time course at least 2 months • Diagnosis • Cerebral spinal fluid • Albuminocytologic disassocation • Nerve conduction studies • Biopsy

  12. Concurrent Illness Variants of CIDP • Several systemic disorders can occur with CIDP • HIV, Hep C • Lymphoma, Myeloma, MGUS • Inflammatory Bowel Disease • Connective Tissue Diseases • Diabetes Mellitus, Thyrotoxicosis • Nephrotic Syndrome • Obligation to search for underlying cause

  13. CIDP Clinical Course • Therapy • IV Immunoglobulin (IVIg) • Repeated infusions, usually 1 course/month • Corticosteroids • Starting dose 100 mg Prednisone per day • Tapered with clinical improvement • Plasmapheresis • Progression with IV IgG or Prednisone • Immunosuppressives • Mycophenolate mofetil, Cyclosporine, Methotrexate

  14. Acquired Ichthyosis • Acquired or Genetic • Acquired usually due to drugs or systemic disease • Rhomboid, or fish-like, scales on the skin • Symmetric, ranges in severity • Primarily affects trunk, limbs, and extensor surfaces • Absence of inflammatory infiltrate with hyperkeratosis is present on skin biopsy

  15. Acquired Icthyosis • Most commonly associated with Hodgkin’s Disease or and non-Hodgkin’s lymphoma • Also seen with • Transitional cell carcinoma, leiomyosarcoma, Kaposi’s Sarcoma, HCC, breast, lung, ovarian cancers • Dermatomyositis • AIDS, HTLV-1 • Sarcoidosis • Thyroid disease • Malnutrition/Malabsorption • Cholesterol-lowering drugs such as Statins and Niacin • No report of association with Strongyloides • Obligation to look for underlying cause

  16. Final Diagnosis • Strongyloides Stercoralis invading stomach • Chronic Active Gastritis • Innumerable sessile colonic Polyps with tubulovillous adenoma and eosinophilic infiltrate

  17. Proposed Pathogenesis Unknown disease process? ? Acquired CIDP Acquired Strongyloides infection Acquired Icthyosis Chronic Illness, Malnutrition Disseminated Infection High Dose Steroids ? Polyp growth GI Bleeding Gastritis Anemia ? Malabsorption

  18. Follow Up • The patient was seen in Neurology clinic 3 weeks ago. • His symptoms have dramatically improved. • The rash is also improving. • He has had no further evidence of GI bleeding. • He will likely begin Azathioprine for his CIDP once the Strongyloides infection is fully resolved.

  19. Thank you!Dr. Martin BlaserDr. Charles Hazzi Dr. Herman YeeDr. Michael MacariDr. Emma RobinsonDr. Jonathan Ralston Dr. Philip TiernoDr. Gerald VillaneuvaDr. Malini SahuDr. Christina Yoon

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