Cyroglobulinemia

Question 1

Types

Answer 1

Type 1: monoclonal

Type 2/3: mixed, most common type II

Question 2

Type 1

Answer 2

MM, Waldenstrom’s

Hyperviscosity +/- thrombosis, cold worsens sx

Question 3

Type 2

Answer 3

Aetiology: HCV infection - 80% are HCV positive, B cell lymphomas, solid organ malignancies
IC-mediated vasculitis with multigrain involvement, can be asx

Question 4

Type 3

Answer 4

Etiology: Autoimmune syndromes: Sjogren’s, SLE, RA, PAN

IC-mediated vasculitis with multigrain involvement, can be asx

Question 5

Other causes

Answer 5

Essential, renal transplant recipient

Infectious in 2/3: HBV, HIV, HAV, EBV, CMV, endocarditis, strep, fungal like coccidiomycosis, malaria, amoebiasis

Question 6

Clinical manifestations

Answer 6

Type 1: hyperviscosity sx: h/a, visual disturbance, livedo, digital ischemia
Type 2/3: vasculitic syndroms
- weakness
- LE purpura, livedo reticularis, leg ulcers
- Joint: migratory arthralgias, symmetric
- GN
- Peripheral neuropathy (polyneuropathy > mononeuritis multiplex)
- Anemia, thrombocytopenia, increase risk of B cell lymphoma
- GI: abdominal pain, HSM, abnormal LFTs

Question 7

Diagnosis

Answer 7

Order cryoglobulins
Cryocrit is quantification of cryoprotein, not always correlate with disease activity
Complement levels to differentiate type 1 from 2/3
- Type 1: complements normal
- T2: decreased C4, variable C3, increased ESR, pos Rhem factor, HCV, HBV, HIV serologies! in all pts with mixed cry
Bx of affected tissue: leukocytoclastic vasculitis in purpuric lesions

Question 8

Treatment

Answer 8

Treat underlying disease
Type 1: plasma exchange if hyper viscosity
Type 2: NSAIDS for control of mild symptoms if normal RF, rituximab or cyclophosphamide for major organ involvement, plasmapheresis in severe, life threatening disease