144 - Cryoglobulinemia and Cryofibrogenemia

Question 1

Circulating immunoglobulins in both serum and plasma that reversibly precipitate or gel upon cold exposure

Answer 1

Cryoglobulins

Question 2

Result form precipitation of fibrinogens on cold exposure and are detectable only in plasma samples, not serum

Answer 2

Cryofibrinogens

Question 3

Cryoglobulinemia and cryofibrogenemia: conditions that lead to microvascular occlusion

Answer 3

Type I cryoglobulinemia

Cryofibrinogenemia

Question 4

Cryoglobulinemia and cryofibrogenemia: conditions that lead to small- and medium-sized vessel vasculitis

Answer 4

Types II and III cryoglobulinemia

Question 5

Consist of a single monoclonal immunoglobulin, typically IgM

Answer 5

Type I cryoglobulins

Question 6

Represent a mixture of 2 Ig components: polyclonal immunoglobulins associated with a monoclonal Ig that exhibits rheumatoid factor activity

Answer 6

Type II cryoglobulins

Question 7

Classic underlying disease in Type II cryoglobulinemia

Answer 7

HCV infection

Question 8

Mixture of polyclonal immunoglobulins or polyclonal Ig-nonimmunoglobulin cryoprecipitates

Answer 8

Type III cryoglobulins

Question 9

Polyclonal IgG associated with a mixture of polyclonal and monoclonal (oligoclonal) IgM

Answer 9

Type II-III cryoglobulins

Question 10

Mixed cryoglobulinemia without an identifiable cause

Answer 10

Essential mixed cryoglobulinemia

Question 11

Essential mixed cryoglobulinemia is more common in _____ with an average onset of _____

Answer 11

Females

54 years

Question 12

The vast majority (>90%) of mixed cryoglobulinemias occurs in association with

Answer 12

Chronic HCV infection

Question 13

Most frequent manifestation of cryoglobulinemia

Answer 13

Cutaneous lesions

Question 14

Infarction, hemorrhagic crust, ulcers, and lesions on the head and oral or nasal mucosa are relatively more common in

Answer 14

Type I cryoglobulinemia

Than in mixed cryoglobulinemia

Question 15

Most common presentation of Type II and III cryoglobulinemic vasculitis

Answer 15

Intermittent orthostatic palpable purpura, frequently observed in the afternoon when highest cryoglobulin concentrations are present

Question 16

Histopathologic hallmark of mixed cryoglobulinemia

Answer 16

Leukocytoclastic vasculitis

Question 17

Most common extracutaneous manifestations in patients with mixed cryoglobulinemic vasculitis

Answer 17

Weakness

Arthralgias or arthritis

Question 18

Metlzer triad

Answer 18

Purpura
Arthralgias
Weakness

Question 19

Most frequently encountered hematologic malignancy in Type I cryoglobulinemia

Answer 19

Non-Hodgkin B-cell lymphoma

Question 20

Type I cryoglobulins tend to precipitate within the first ______, where Type III cryoglobulins may require

Answer 20

24 hours

7 days

Question 21

A cryocit of at least _____% is considered positive

Answer 21

2

Question 22

Reference serum viscosity is between 1.4 and 1.8, whereas most symptomatic patients have values between

Answer 22

5 and 8

Question 23

Typical pattern of hypocomplementemia in mixed cryoglobulinemia

Answer 23

Low or undetectable C4

Normal or relatively normal C3 levels

Question 24

Patients with non-HCV mixed cryoglobulinemia have overall (lower/higher) mortality rate than their HCV-positive counterparts

Answer 24

Higher

Question 25

Most common cause of death in HCV patients with mixed cryoglobulinemia, followed by complications related to liver and cardiac involvement

Answer 25

Infection

Question 26

Organs most vulnerable to acute severe complications directly caused by cryoglobulins

Answer 26

Nervous system | Kidneys

Question 27

Treatment of choice for type I cryoglobulinemia

Answer 27

Etiologic treatment

Question 28

Patients with mild to moderate HCV-associated cryoglobulinemic vasculitis without major organ failure are best treatment with

Answer 28

PEGylated interferon alpha-2b Ribavirin Current gold standard

Question 29

Applicability of _____ in renal insufficiency is reduced because of drug accumulation

Answer 29

Ribavirin

Question 30

Side of effect of ribavirin requirng pausing or dosing adjustments

Answer 30

Hemolytic anemia

Question 31

Preferred therapeutic regimen in patients withHCV-associated severe or refractory/relapsing disease, renal involvement, and in those whom rapid clinical response is needed

Answer 31

Rituximab combined with Peg-IFN-alpha/ribavirin

Question 32

Should not be used in patients with overt skin ulceration because of interference with wound healing

Answer 32

Rituximab

Question 33

A recent report has pointed to a severe complication with worsening of cryoglobulinemic vasculitis syndrome in patients with high baseline levels of mixed cryoglobulins receiving

Answer 33

Rituximab

Question 34

_____% of healthy persons may have demonstrable amounts of cryofibrinogen

Answer 34

2-9

Question 35

Patients with cryofibrinogenemia are usually diagnosed between the _____ decades of life, with a _____ predilection

Answer 35

Fifth and seventh | Female

Question 36

Most frequent clinical presentation of cryofibrinogenemia

Answer 36

Palpable purpura with underlying leukocytoclastic vasculitis

Question 37

Pathogenesis of cryofibrinogenemia: precipitation of patients' native fibrinogen or fibrin byproducts in (serum/plasma), but not in (serum/plasma)

Answer 37

Plasma | Serum

Question 38

Most frequently associated disorders with secondary cryofibrinogenemia

Answer 38

Malignancy Infections Connective tissue disease Autoimmune disease

Question 39

The warm blood specimen should be anticoagulated with citrate, EDTA, or oxalate, but not _____, which nonspecifically precipitates fibrinogen and may result in a false positive result

Answer 39

Heparin

Question 40

Histologic features of cryofibrinogenemia irrespective of the anatomic site

Answer 40

Occlusive thrombotic diathesis comprising eosinophilic refractile deposits within vessel lumina with extension into the vessel intima

Question 41

Typical complications of cryofibrinogenemia due to thrombotic events

Answer 41

Gangrene Septicemia Leg amputation

Question 42

Synthetic derivative of testosterone with substantial fibrinolytic properties Has been effective for essential cryofibrinogenemia

Answer 42

Oral stanozolol (2-4 mg twice daily)

Question 43

Has a more rapid onset of action than stanozolol

Answer 43

Streptokinase (sometimes in combination with streptodornase), given intravenously (25,000-200,000 U/d)

Question 44

Other combination treatments for cryofibrinogenemia

Answer 44

Colchicine (0.6 mg twice daily) in combination with high-dose pentoxifylline (800 mg 3 times daily) Combination of glucocorticoids (prednisone 10-60 mg/d) with low-dose aspirin, or other immunosuppressive agents (azathioprine 150 mg/d or chlorambucil 10 mg/d)