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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 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
A diagnostic test was performed: Endoscopy and Colonoscopy with biopsies
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
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.
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
Strongyloides Life Cycle parthenogenesis FECES SOIL infective larvae
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”
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
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
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
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
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
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
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
Final Diagnosis • Strongyloides Stercoralis invading stomach • Chronic Active Gastritis • Innumerable sessile colonic Polyps with tubulovillous adenoma and eosinophilic infiltrate
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
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.
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