Clinical Pathological Case Conference -

AnswerKristin Remus, D.O.Chief ResidentNYU School of Medicine, Internal MedicineAugust 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

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 - negativeHu immunoreactivity – negativeAnti-ganglioside IgM <1:800Anti-ganglioside IgG <1:100

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

infective larvae

SOIL

FECES

parthenogenesis

Strongyloides Life Cycle

Strongyloides Stercoralis Clinical Presentation

Skin larva currens

GI tract Cramps, diarrhea Malabsorption Rarely massive

hemorrhage Immunosuppressed

Fever Lungs

larvae in sputum

Many fatalities reportedCutaneous 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

Acquired CIDP

Proposed Pathogenesis

Acquired Icthyosis

?

Chronic Illness,Malnutrition

High DoseSteroids

AcquiredStrongyloides infection

GI BleedingGastritisAnemia

? Malabsorption

Disseminated Infection

? Polyp growth

Unknown disease process?

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