Clinical Pathological Case Conference - Answer

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Clinical Pathological Case Conference - Answer

• Kristin Remus, D.O.
• Chief Resident
• NYU School of Medicine, Internal Medicine
• August 8, 2008

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Radiology

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

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A diagnostic test was performed Endoscopy and
Colonoscopy with biopsies
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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

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Additional Lab Results
Purkinje Cell Ab - negative Hu immunoreactivity
negative Anti-ganglioside IgM lt1800 Anti-ganglios
ide IgG lt1100
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 lt0.5 (lt5) CA-125
U/mL 14.2 (lt35) 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.
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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

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Strongyloides Life Cycle
parthenogenesis
FECES
SOIL
infective larvae
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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
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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

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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

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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

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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

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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

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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

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Acquired Icthyosis

• Most commonly associated with Hodgkins Disease
  or and non-Hodgkins lymphoma
• Also seen with
• Transitional cell carcinoma, leiomyosarcoma,
  Kaposis 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

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Final Diagnosis

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

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