Transcript Clinical Pathological Case Conference

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
Iron ug/dL 70 (42-146)
TIBC ug/dL 189 (250450)
Ferritin ng/mL 186.7
(22-322)
Retic % 3.77 (0.5-1.55)
Retic Index 2%
Purkinje Cell Ab - negative
Hu immunoreactivity – negative
Anti-ganglioside IgM <1:800
Anti-ganglioside IgG <1:100
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


GI tract




larva currens
Cramps, diarrhea
Malabsorption
Rarely massive
hemorrhage
Immunosuppressed


Fever
Lungs


larvae in sputum
Many fatalities reported
Cutaneous larva currens, “racing larva”
Stronglyoides Infection

Immunosuppresion



Hyperinfection


Disseminated infection
Treatment


Steroids may mimic endogenous parasitic-derived
regulatory hormone
More eggs produced in the presence of exogenous steroids
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)


Corticosteroids



Starting dose 100 mg Prednisone per day
Tapered with clinical improvement
Plasmapheresis


Repeated infusions, usually 1 course/month
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
?
Acquired CIDP
Unknown disease
process?
Acquired
Strongyloides infection
Acquired Icthyosis
Chronic Illness,
Malnutrition
High Dose
Steroids
Disseminated
Infection
? 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.
Thank you!
Dr. Martin Blaser
Dr. Charles Hazzi
Dr. Herman Yee
Dr. Michael Macari
Dr. Emma Robinson
Dr. Jonathan Ralston
Dr. Philip Tierno
Dr. Gerald Villaneuva
Dr. Malini Sahu
Dr. Christina Yoon