cpc 8 08 part 1a

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 : 

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

Slide 6: 

A diagnostic test was performed: Endoscopy and Colonoscopy with biopsies

Further Studies : 

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 : 

Additional Lab Results 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. Purkinje Cell Ab - negative Hu immunoreactivity – negative Anti-ganglioside IgM <1:800 Anti-ganglioside IgG <1:100

Strongyloides Stercoralis : 

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

Slide 14: 

infective larvae SOIL FECES parthenogenesis Strongyloides Life Cycle

Strongyloides Stercoralis : 

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 : 

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) : 

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 : 

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 : 

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 : 

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 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 : 

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 : 

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

Slide 24: 

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

Follow Up : 

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.

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 : 

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