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FD9 > 144 - Cryoglobulinemia and Cryofibrogenemia > Flashcards

144 - Cryoglobulinemia and Cryofibrogenemia Flashcards

1
Q

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

A

Cryoglobulins

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2
Q

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

A

Cryofibrinogens

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3
Q

Cryoglobulinemia and cryofibrogenemia: conditions that lead to microvascular occlusion

A

Type I cryoglobulinemia

Cryofibrinogenemia

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4
Q

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

A

Types II and III cryoglobulinemia

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5
Q

Consist of a single monoclonal immunoglobulin, typically IgM

A

Type I cryoglobulins

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6
Q

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

A

Type II cryoglobulins

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7
Q

Classic underlying disease in Type II cryoglobulinemia

A

HCV infection

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8
Q

Mixture of polyclonal immunoglobulins or polyclonal Ig-nonimmunoglobulin cryoprecipitates

A

Type III cryoglobulins

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9
Q

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

A

Type II-III cryoglobulins

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10
Q

Mixed cryoglobulinemia without an identifiable cause

A

Essential mixed cryoglobulinemia

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11
Q

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

A

Females

54 years

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12
Q

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

A

Chronic HCV infection

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13
Q

Most frequent manifestation of cryoglobulinemia

A

Cutaneous lesions

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14
Q

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

A

Type I cryoglobulinemia

Than in mixed cryoglobulinemia

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15
Q

Most common presentation of Type II and III cryoglobulinemic vasculitis

A

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

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16
Q

Histopathologic hallmark of mixed cryoglobulinemia

A

Leukocytoclastic vasculitis

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17
Q

Most common extracutaneous manifestations in patients with mixed cryoglobulinemic vasculitis

A

Weakness

Arthralgias or arthritis

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18
Q

Metlzer triad

A

Purpura
Arthralgias
Weakness

19
Q

Most frequently encountered hematologic malignancy in Type I cryoglobulinemia

A

Non-Hodgkin B-cell lymphoma

20
Q

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

A

24 hours

7 days

21
Q

A cryocit of at least _____% is considered positive

A

2

22
Q

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

A

5 and 8

23
Q

Typical pattern of hypocomplementemia in mixed cryoglobulinemia

A

Low or undetectable C4

Normal or relatively normal C3 levels

24
Q

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

A

Higher

25
Q

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

A

Infection

26
Q

Organs most vulnerable to acute severe complications directly caused by cryoglobulins

A

Nervous system

Kidneys

27
Q

Treatment of choice for type I cryoglobulinemia

A

Etiologic treatment

28
Q

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

A

PEGylated interferon alpha-2b
Ribavirin

Current gold standard

29
Q

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

A

Ribavirin

30
Q

Side of effect of ribavirin requirng pausing or dosing adjustments

A

Hemolytic anemia

31
Q

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

A

Rituximab combined with Peg-IFN-alpha/ribavirin

32
Q

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

A

Rituximab

33
Q

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

A

Rituximab

34
Q

_____% of healthy persons may have demonstrable amounts of cryofibrinogen

A

2-9

35
Q

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

A

Fifth and seventh

Female

36
Q

Most frequent clinical presentation of cryofibrinogenemia

A

Palpable purpura with underlying leukocytoclastic vasculitis

37
Q

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

A

Plasma

Serum

38
Q

Most frequently associated disorders with secondary cryofibrinogenemia

A

Malignancy
Infections
Connective tissue disease
Autoimmune disease

39
Q

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

A

Heparin

40
Q

Histologic features of cryofibrinogenemia irrespective of the anatomic site

A

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

41
Q

Typical complications of cryofibrinogenemia due to thrombotic events

A

Gangrene
Septicemia
Leg amputation

42
Q

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

A

Oral stanozolol (2-4 mg twice daily)

43
Q

Has a more rapid onset of action than stanozolol

A

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

44
Q

Other combination treatments for cryofibrinogenemia

A

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)

FD9 (108 decks)

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