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Fitzpatrick's Dermatology, 9th Edition (Chapter-based Decks) > 144: Cryoglobulinemia & Cryofibrinogenemia > Flashcards

144: Cryoglobulinemia & Cryofibrinogenemia Flashcards

1
Q

What are cryoglobulins and how do they behave in serum or plasma?

A

Cryoglobulins are circulating immunoglobulin complexes found in plasma or serum that reversibly precipitate in cold temperatures. They can be asymptomatic or cause a clinical syndrome involving the skin, such as purpura or ulceration.

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2
Q

What are the classifications of cryoglobulinemia according to Brouet?

A

Cryoglobulinemia is classified into three types:

  1. Type I: Consists of a single monoclonal immunoglobulin (typically IgG or light chain).
  2. Type II: Mixed cryoglobulinemia with polyclonal immunoglobulin (typically IgG) and monoclonal components.
  3. Type III: Composed of purely polyclonal immunoglobulin, often associated with chronic inflammatory states.
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3
Q

What is the epidemiology of mixed cryoglobulinemia?

A

Mixed cryoglobulinemia has an incidence of 1 to 7 in 100,000, with a higher prevalence in Southern Europe due to the geographic distribution of HCV. The average age at onset is 54 years, and it is associated with chronic HCV infection in over 90% of cases.

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4
Q

What are the clinical associations of essential mixed cryoglobulinemia?

A

Essential mixed cryoglobulinemia is associated with:
- Infectious diseases (75%)
- Autoimmune diseases (24%)
- Hematologic diseases (7%)
It occurs without an identifiable cause and has a male-to-female ratio of 1:3.

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5
Q

What are the clinical implications of symptomatic cryoglobulinemic vasculitis syndrome in HCV patients?

A

Symptomatic cryoglobulinemic vasculitis syndrome occurs in 2% to 5% of patients with chronic HCV infection. This condition is characterized by immune complex-mediated vasculitis affecting the skin, neural, and renal tissues, highlighting the need for careful monitoring in HCV patients.

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6
Q

What is cryoglobulinemia?

A

The presence of circulating immunoglobulin complexes in plasma or serum that reversibly precipitate in cold temperatures.

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7
Q

What is the average age at onset for essential mixed cryoglobulinemia?

A

54 years.

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8
Q

What is the prevalence of mixed cryoglobulinemia?

A

1 to 7 in 100,000.

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9
Q

What is the association of mixed cryoglobulinemia with chronic HCV infection?

A

The vast majority of mixed cryoglobulinemias occurs in association with chronic HCV infection (>90%).

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10
Q

What are the clinical implications of cryoglobulinemia?

A

It can be asymptomatic or cause a clinical syndrome involving the skin, such as purpura and ulceration.

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11
Q

What is the significance of Type I cryoglobulins?

A

Type I cryoglobulins consist of a single monoclonal immunoglobulin and are associated with hematologic malignancies.

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12
Q

What is the difference between Type II and Type III cryoglobulins?

A

Type II cryoglobulins are mixed and associated with chronic inflammatory states, while Type III cryoglobulins are associated with chronic HCV or connective tissue disease.

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13
Q

What percentage of chronically HCV-infected subjects develop cryoglobulinemia?

A

30%, which can increase to 90% in patients with longstanding disease.

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14
Q

What is the most common cause of mixed cryoglobulinemia?

A

The most common cause is chronic HCV infection, associated with >90% of cases.

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15
Q

What are the clinical implications of Type I cryoglobulinemia and how does it differ from Types II and III?

A

Type I cryoglobulinemia consists of a monoclonal immunoglobulin (typically IgG or light chain) and can lead to occlusive vasculopathy. In contrast, Types II and III are referred to as mixed cryoglobulinemias, often associated with chronic inflammatory states and can involve polyclonal immunoglobulins.

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16
Q

What is the epidemiological significance of mixed cryoglobulinemia in relation to chronic HCV infection?

A

Mixed cryoglobulinemia has a notable epidemiological significance, particularly in Southern Europe, where it is linked to the geographic distribution of HCV.

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17
Q

How does the presence of cryoglobulins in a patient’s serum relate to the clinical presentation of cryoglobulinemia?

A

The presence of cryoglobulins in a patient’s serum can be asymptomatic or lead to occlusive vasculopathy, known as cryoglobulinemic syndrome.

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18
Q

What are the most common cutaneous findings in patients with cryoglobulinemic vasculitis?

A

The most common cutaneous findings include:
- Type I: Often asymptomatic; may present with infarction, hemorrhagic crusts, ulcers, and lesions on the head and oral or nasal mucosa.
- Type II and III: Cryoglobulinemic vasculitis with intermittent orthostatic palpable purpura, sporadic isolated petechiae or erythematous macules.

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19
Q

What are the non-cutaneous findings associated with mixed cryoglobulinemias?

A

The most common extracutaneous manifestations include:
- Weakness
- Arthralgias
- Arthritis
Additionally, the Meltzer Triad consists of:
- Purpura
- Arthralgias
- Weakness

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20
Q

What renal complications are associated with cryoglobulinemic vasculitis?

A

Renal complications include:
- Incidence: 5-60% of patients
- Common findings:
- Hematuria with or without renal insufficiency (41%)
- Isolated proteinuria
- Nephrotic syndrome (21%)

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21
Q

What neurological symptoms are associated with mixed cryoglobulinemia?

A

Neurological symptoms can include:
- Sensory peripheral nervous system issues secondary to epineural vasculitis
- Paresthesias with burning symptoms in the lower limbs.

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22
Q

What musculoskeletal symptoms are prevalent in patients with cryoglobulinemia?

A

Musculoskeletal symptoms include:
- 70% of patients experience arthralgias and myalgias, predominantly in Type II and III.

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23
Q

What pulmonary complications can arise in patients with cryoglobulinemia?

A

Pulmonary complications can include:
- 40% of patients may experience dyspnea, cough, or pleuritic pain.

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24
Q

What is the most frequent manifestation of patients with mixed cryoglobulinemias?

A

Weakness, arthralgias, or arthritis.

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25
Q

What is a common neurologic manifestation in patients with mixed cryoglobulinemia?

A

Peripheral neuropathy, which can occur in up to 80% of patients.

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26
Q

What is the Meltzer triad associated with mixed cryoglobulinemias?

A

Purpura, arthralgias, and weakness.

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27
Q

What are the pulmonary manifestations seen in patients with mixed cryoglobulinemia?

A

Dyspnea, cough, or pleuritic pain, with pulmonary function tests showing small airways disease.

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28
Q

What is the hallmark histopathologic finding in mixed cryoglobulinemia?

A

Livedo vasculitis (LCV).

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29
Q

What is a significant renal finding in patients with mixed cryoglobulinemia?

A

Hematuria with or without renal insufficiency.

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30
Q

A patient presents with purpura, arthralgias, and weakness. What is the likely diagnosis and what is this triad called?

A

The likely diagnosis is mixed cryoglobulinemia, and the triad is called Meltzer’s triad.

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31
Q

A patient with cryoglobulinemia presents with renal involvement. What is the most common renal pathology associated with mixed cryoglobulinemia?

A

Membranoproliferative glomerulonephritis (MPGN) is the most common renal pathology associated with mixed cryoglobulinemia.

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32
Q

What is the hallmark histopathologic finding in mixed cryoglobulinemia?

A

Leukocytoclastic vasculitis (LCV) is the hallmark histopathologic finding in mixed cryoglobulinemia.

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33
Q

What is the most common extracutaneous manifestation of mixed cryoglobulinemias?

A

The most common extracutaneous manifestations are weakness, arthralgias, or arthritis.

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34
Q

What is the most common pulmonary finding in patients with cryoglobulinemia?

A

The most common pulmonary findings are dyspnea, cough, or pleuritic pain.

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35
Q

What is the characteristic fundoscopic finding in Type I cryoglobulinemia?

A

The characteristic finding is ‘sausaging,’ which refers to retinal venous engorgement.

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36
Q

What is the most common cutaneous finding in cryoglobulinemia?

A

The most common cutaneous finding is palpable purpura.

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37
Q

What is the most common neurologic manifestation of mixed cryoglobulinemia?

A

The most common neurologic manifestation is sensory peripheral neuropathy.

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38
Q

What is the most common renal complication in mixed cryoglobulinemia?

A

The most common renal complication is membranoproliferative glomerulonephritis (MPGN).

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39
Q

What are the most common cutaneous findings in patients with Type I cryoglobulinemic vasculitis?

A
  • Often asymptomatic
  • Infarction, hemorrhagic crusts, ulcers, and lesions on the head and oral or nasal mucosa (more common in Type I than mixed cryoglobulinemia)
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40
Q

What are the common non-cutaneous findings in patients with mixed cryoglobulinemias?

A
  • Weakness
  • Arthralgias
  • Arthritis
  • MELTZER TRIAD (25-30% of patients): Purpura, arthralgias, weakness
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41
Q

What percentage of patients with mixed cryoglobulinemia experience renal involvement, and what are the common renal manifestations?

A
  • Incidence: 5-60%
  • Common manifestations include:
    • Hematuria with or without renal insufficiency (41%)
    • Isolated proteinuria
    • Nephrotic syndrome (21%)
    • Acute nephritic syndrome
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42
Q

What neurological symptoms are associated with mixed cryoglobulinemia, and what is the prevalence of peripheral neuropathy in these patients?

A
  • Neurological symptoms include:
    • Sensory peripheral nervous system issues secondary to epineural vasculitis
    • Paresthesias with burning symptoms in the lower limbs
    • Peripheral neuropathy in up to 80% of patients with mixed cryoglobulinemia (prevalence of only 5% to 45% in symptom-based studies)
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43
Q

What are the pulmonary manifestations observed in patients with mixed cryoglobulinemia?

A
  • 40% of patients experience:
    • Dyspnea, cough, or pleuritic pain
    • Pulmonary function tests indicating small airways disease
    • CXR may show interstitial infiltrates or signs of subclinical alveolitis
    • Uncommon pulmonary vasculitis and severe pulmonary disease (e.g., BOOP)
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44
Q

What are the main types of cryoglobulinemia and their associations with HCV infection?

A
  • Type I: Associated with hematologic disorders, particularly Non-Hodgkin B-cell lymphoma.
  • Essential mixed cryoglobulinemia: Occurs without underlying disease in a minority of patients.
  • Mixed cryoglobulinemia:
    • Serum anti-HCV antibodies and/or HCV RNA found in 70-100% of patients.
    • Type II is more strongly associated with HCV than Type III (90% vs 70%).
    • Cryoglobulins are found in 55-90% of patients with longstanding HCV infection, with overt syndrome developing in only 2-5% of these cases.
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45
Q

What are the clinical features that suggest a diagnosis of cryoglobulinemia?

A

The possibility of diagnosis is suggested by:
- Purpura of the distal extremities
- Acral cyanosis
- Necrosis

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46
Q

What laboratory testing procedures are recommended for diagnosing cryoglobulinemia?

A

Laboratory testing should include:
1. Cryoglobulin testing: Establishing the diagnosis when positive.
2. Complete blood count: Including differential.
3. Serum biochemistry: To assess liver disease or other conditions.
4. Serum must be obtained in warm tubes at 37°C in the absence of anticoagulants.
5. Temperature should be kept at 37°C until laboratory processing.

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47
Q

What are the associations of HCV with cryoglobulinemia and related conditions?

A

HCV is associated with:
- Longstanding infection
- Old age
- Type II cryoglobulins
- Higher cryoglobulin levels
- Clonal B-cell expansion (t(14;18) translocation with bcl-2 rearrangement)
- HCV-associated cryoglobulinemic vasculitis syndrome: Increased mortality risk, especially in patients with Sjögren syndrome and SLE.

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48
Q

What are the common hematologic disorders associated with Type I cryoglobulinemia?

A

Type I cryoglobulinemia is commonly associated with:
- Hematologic disorders
- Non-Hodgkin B-cell lymphoma, which is the most frequently encountered hematologic malignancy.

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49
Q

What is the relationship between HCV infection duration and the presence of cryoglobulins?

A

The presence of cryoglobulins increases with the duration of HCV infection:
- Cryoglobulins are found in 55% to 90% of patients with longstanding infection.
- Overt cryoglobulinemic syndrome develops in only 2% to 5% of these cases.

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50
Q

What factors may predispose individuals to extrahepatic systemic manifestations of cryoglobulinemia?

A

Individuals with HLA-DR11 or HLA-DR6 may be predisposed to extrahepatic systemic manifestations of cryoglobulinemia.

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51
Q

What are the mandatory laboratory tests for diagnosing cryoglobulinemia?

A

Mandatory laboratory tests for diagnosing cryoglobulinemia include:
1. Cryoglobulin testing (should be positive)
2. Complete blood count, including differential
3. Serum BUN
4. Liver function tests
5. ANA, SSA, and SSB (to rule out associated conditions)
6. Consider histology for further evaluation if necessary.

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52
Q

What clinical features suggest a diagnosis of cryoglobulinemia?

A

The possibility of diagnosing cryoglobulinemia is suggested by:
- Presence of purpura of the distal extremities
- Acral cyanosis
- Necrosis

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53
Q

What is the significance of Type II cryoglobulinemia in relation to HCV?

A

Type II cryoglobulinemia is more strongly associated with HCV than Type III, with a prevalence of 90% compared to 70% respectively.

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54
Q

What is the most frequently encountered hematologic malignancy associated with Type I cryoglobulinemia?

A

Non-Hodgkin B-cell lymphoma.

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55
Q

What percentage of cryoglobulinemic patients have serum anti-HCV antibodies and/or HCV RNA?

A

70-100%.

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56
Q

How is Type II cryoglobulinemia related to HCV?

A

It is more strongly associated with HCV than Type III (90% vs 70%).

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57
Q

What factors increase the presence of cryoglobulins in patients with HCV infection?

A

Duration of HCV infection; cryoglobulins are found in 55% to 90% of patients with longstanding infection.

58
Q

What are the potential systemic manifestations of cryoglobulinemia?

A

Extrahepatic systemic manifestations.

59
Q

What is the significance of HLA-DR11 or HLA-DR6 in cryoglobulinemia?

A

They may predispose to extrahepatic systemic manifestations of cryoglobulinemia.

60
Q

What laboratory testing is mandatory for diagnosing cryoglobulinemia?

A

Cryoglobulin testing, complete blood count, and specific serological tests.

61
Q

What symptoms suggest the possibility of cryoglobulinemia diagnosis?

A

Purpura of the distal extremities or acral cyanosis and necrosis.

62
Q

What is the recommended temperature for serum samples during cryoglobulin testing?

A

37 °C until laboratory processing.

63
Q

What is the association between Sjögren syndrome and cryoglobulinemia?

A

16% of patients with primary Sjögren had cryoglobulins, and 56% had cryoglobulinemic syndrome.

64
Q

A patient with longstanding HCV infection develops cryoglobulinemic vasculitis. What type of cryoglobulinemia is most strongly associated with HCV?

A

Type II cryoglobulinemia is most strongly associated with HCV.

65
Q

What laboratory test is essential for diagnosing cryoglobulinemia, and what temperature must the serum be kept at during processing?

A

The serum must be obtained in warm tubes at 37°C and kept at this temperature until laboratory processing.

66
Q

A patient with cryoglobulinemia presents with acral cyanosis and necrosis. What is the likely diagnosis?

A

The likely diagnosis is cryoglobulinemia, as suggested by the presence of acral cyanosis and necrosis.

67
Q

What is the most frequent hematologic malignancy associated with Type I cryoglobulinemia?

A

Non-Hodgkin B-cell lymphoma is the most frequently encountered hematologic malignancy.

68
Q

What are the key characteristics of Type I cryoglobulinemia and its association with hematologic disorders?

A

Type I cryoglobulinemia is characterized by:
- Hematologic disorders such as Non-Hodgkin B-cell lymphoma, which is the most frequently encountered hematologic malignancy.
- Serum anti-HCV antibodies and/or HCV RNA are present in 70-100% of cryoglobulinemic patients.
- It is more strongly associated with HCV than Type II cryoglobulinemia (90% vs 70%).

69
Q

What are the clinical implications of HLA-DR11 or HLA-DR6 in cryoglobulinemia?

A

HLA-DR11 or HLA-DR6 may predispose patients to:
- Extrahepatic systemic manifestations of cryoglobulinemia, which can complicate the clinical picture and management of the disease.

70
Q

What laboratory testing protocols should be followed for diagnosing cryoglobulinemia?

A

Laboratory testing for cryoglobulinemia should include:
1. Cryoglobulin testing during active flares, repeated several weeks apart.
2. Serum must be obtained in warm tubes at 37°C in the absence of anticoagulants.
3. Temperature should be kept at 37°C until laboratory processing.

71
Q

What are the associations of HCV-related cryoglobulinemic vasculitis syndrome?

A

HCV-associated cryoglobulinemic vasculitis syndrome is associated with:
- Longstanding infection
- Old age
- Type II cryoglobulins
- Higher cryoglobulin levels
- Clonal B-cell expansion (t(14;18) translocation with bcl-2 rearrangement).

72
Q

What is the typical time frame for Type I cryoglobulins to precipitate after serum separation?

A

Type I cryoglobulins tend to precipitate within the first 24 hours at concentrations greater than 5 mg/mL.

73
Q

What is the significance of a cryocrit of ≥ 2% in cryoglobulin testing?

A

A cryocrit of ≥ 2% is considered to be positive for cryoglobulins, indicating their presence in the serum.

74
Q

What are the common complications associated with Type I cryoglobulinemia?

A

Complications of Type I cryoglobulinemia include:
1. Acute vaso-occlusive crisis due to increased serum viscosity, leading to:
- Acute acral ischemia
- Cerebral ischemia
- Renal ischemia, causing stroke or acute renal failure
2. Myeloma kidney
3. Advanced stages may lead to:
- Immunosuppression
- Infection and sepsis
- Coagulation disorders.

75
Q

What is the prognosis for patients with cryoglobulinemic vasculitis (Type II or III)?

A

Patients with cryoglobulinemic vasculitis (Type II or III) experience:
- Increased morbidity and mortality
- 10-year survival rates significantly lower than the normal population due to complications such as renal involvement, liver and cardiac disease, and myeloproliferative disorders.

76
Q

What is the relationship between HCV-associated cryoglobulinemic vasculitis and antiviral therapy?

A

In HCV-associated cryoglobulinemic vasculitis, response to antiviral therapy reduces the mortality rate among patients.

77
Q

What are the clinical features of non-HCV mixed cryoglobulinemia?

A

Non-HCV mixed cryoglobulinemia is characterized by:
- Lower gammaglobulin levels
- Increased frequency of renal involvement
- 4-fold increased risk of developing B-cell lymphoma
- Overall higher mortality rate compared to HCV-positive counterparts.

78
Q

What is the expected time frame for Type I cryoglobulins to precipitate after serum separation and storage at 4°C?

A

Type I cryoglobulins tend to precipitate within the first 24 hours at concentrations greater than 5 mg/mL.

79
Q

What complications are associated with cryoglobulinemia?

A

Complications include renal involvement, liver and cardiac disease, cardiovascular complications, and myeloproliferative disorders.

80
Q

What is the relationship between non-HCV mixed cryoglobulinemia and renal involvement?

A

In non-HCV mixed cryoglobulinemia, there is lower gammaglobulin levels, increased frequency of renal involvement, a 4-fold increased risk of developing B-cell lymphoma, and an overall higher mortality rate compared to HCV-positive counterparts.

81
Q

What is the typical time frame for Type I cryoglobulins to precipitate after serum separation?

A

Within the first 24 hours at concentrations greater than 5 mg/mL.

82
Q

What is the significance of a cryocrit of 2% or greater?

A

It is considered positive for cryoglobulins.

83
Q

What does the Oswald-type viscometer measure in relation to cryoglobulinemia?

A

Serum viscosity.

84
Q

What percentage of patients may experience hypocomplementemia in cryoglobulinemia?

A

Up to 90%.

85
Q

What is a common complication associated with Type I cryoglobulinemia?

A

Acute vaso-occlusive crisis caused by increased serum viscosity.

86
Q

What is the prognosis for patients with cryoglobulinemic vasculitis?

A

Increased morbidity and mortality, with significantly lower 10-year survival rates than the normal population.

87
Q

What is the most common cause of death in HCV patients with mixed cryoglobulinemia?

A

Infection, followed by liver and cardiac involvement.

88
Q

What is the relationship between renal involvement and prognosis in cryoglobulinemic vasculitis?

A

Renal involvement is a poor prognostic sign, with 15% progressing to end-stage renal disease (ESRD).

89
Q

What is the expected prognosis for Type I cryoglobulinemia?

A

A favorable prognosis is expected when control of the underlying disease is achieved.

90
Q

What is the risk associated with non-HCV mixed cryoglobulinemia?

A

Increased frequency of renal involvement and a four-fold increased risk of developing B-cell lymphoma.

91
Q

A patient with cryoglobulinemia has a cryocrit of 3%. Is this considered positive, and does it correlate with disease severity?

A

A cryocrit ≥2% is considered positive, but it does not correlate with disease severity.

92
Q

What is the significance of a sudden increase in C4 levels in a patient with cryoglobulinemia?

A

A sudden increase in C4 levels to abnormally high levels may indicate the development of a B-cell lymphoma.

93
Q

What is the primary difference in cryoprecipitation time between Type I and Type III cryoglobulins?

A

Type I cryoglobulins tend to precipitate within the first 24 hours, while Type III requires up to 7 days.

94
Q

What are the common complications associated with Type I cryoglobulinemia?

A

Common complications include acute vaso-occlusive crisis, myeloma kidney, and immunosuppression leading to infection and coagulation disorders.

95
Q

What laboratory findings are indicative of hypocomplementemia in patients with cryoglobulinemia?

A

Low levels of C3 and C4 complement proteins, with a sudden increase in C4 indicating possible B-cell lymphoma.

96
Q

What are the three main management strategies for cryoglobulinemia?

A
  1. Etiologic: containment of the underlying disease 2. Pathophysiologic: reducing the production of cryoglobulins 3. Symptomatic: reducing the cryoglobulin burden.
97
Q

What is the treatment of choice for Type I cryoglobulinemia?

A

Etiologic treatment, which may include chemotherapy for the underlying hematologic disease. Plasma exchange and cryofiltration are recommended for severe symptomatic hyperviscosity syndrome.

98
Q

What antiviral treatments are recommended for HCV-associated Type II and III cryoglobulinemia in patients without major organ failure?

A

PEGylated interferon a-2b and ribavirin.

99
Q

What is the role of Rituximab in the treatment of severe cryoglobulinemia?

A

Rituximab depletes 95% of B cell population, reducing cryoglobulin levels significantly.

100
Q

What are the typical clinical features of cryofibrinogenemia?

A

Thrombosis and thrombotic phenomena of skin and viscera, often life-threatening.

101
Q

What is the epidemiology of cryofibrinogenemia?

A

Cryofibrinogenemia is rare but probably underestimated; 2% to 9% of healthy persons may have demonstrable amounts.

102
Q

What is cryofibrinogenemia and how is it classified?

A

Cryofibrinogenemia results from cryoprecipitation of native fibrinogen or fibrin by-products in plasma. It is classified as essential or secondary.

103
Q

What are the common noncutaneous findings in patients with cryofibrinogenemia?

A

Fever, malaise, vascular involvement, thrombotic events, and high cryofibrinogen plasma concentration.

104
Q

What are the conditions associated with secondary cryofibrinogenemia?

A

Malignancy, infections, connective tissue diseases, and autoimmune diseases.

105
Q

What are the common clinical features associated with cryofibrinogenemia?

A

Common clinical features include:

  • Cutaneous findings: Temporal association between cold exposure and onset of symptoms, palpable purpura with underlying leucocytoclastic vasculitis, painful ulcerations, livedo racemosa, Raynaud phenomenon, segmental swelling, and cold urticaria.
  • Noncutaneous findings: Fever and malaise, vascular, kidney, musculoskeletal, and/or neuronal involvement, thrombotic events, nephritic or nephrotic syndrome, arthralgia, myalgia, multineuritis.
106
Q

What are the conditions associated with secondary cryofibrinogenemia?

A

Conditions associated with secondary cryofibrinogenemia include: Malignancy, infections, connective tissue diseases, and autoimmune diseases.

107
Q

How is cryofibrinogen classified?

A

Cryofibrinogen is classified as primary (essential or idiopathic) or secondary.

108
Q

What is required for the diagnosis of primary cryofibrinogenemia?

A

Presence of cryofibrinogens in plasma, absence of cryoglobulins, and one or more compatible clinical features.

109
Q

What are the potential complications associated with high cryofibrinogen plasma concentration?

A

High cryofibrinogen plasma concentration is a predisposing factor for various thrombotic events, which can lead to:

  • Cerebrovascular accidents (CVA)
  • Myocardial infarction (MI)
  • Limb and bowel ischemia or infarction
  • Pulmonary emboli.
110
Q

What age group is most commonly affected by cryofibrinogenemia?

A

Between the fifth and seventh decades of life.

111
Q

What is the most frequent clinical presentation of cryofibrinogenemia?

A

Palpable purpura with underlying leucocytoclastic vasculitis.

112
Q

What are common noncutaneous findings in cryofibrinogenemia?

A

Fever, malaise, and various thrombotic events.

113
Q

What is a predisposing factor for thrombotic events in cryofibrinogenemia?

A

High cryofibrinogen plasma concentration.

114
Q

What is the significance of cold exposure in cryofibrinogenemia?

A

It is associated with the onset of symptoms and can lead to cold-induced thromboses.

115
Q

What are the main clinical manifestations of cryofibrinogenemia?

A

Purpura, skin necrosis, and arthralgia.

116
Q

What is the primary composition of cryofibrinogen?

A

Cryofibrinogen is composed of fibrinogen, fibrin, fibronectin, and/or fibrin degradation products.

117
Q

What are the common cutaneous findings associated with cryofibrinogenemia?

A

Common cutaneous findings include:

  • Temporal association with cold exposure and onset of symptoms.
  • Palpable purpura with underlying leucocytoclastic vasculitis.
  • Other manifestations: painful ulcerations, livedo racemosa, Raynaud phenomenon, segmental swelling, lower extremity nodules, and cold urticaria.
118
Q

What is the histological characteristic of cryofibrinogen precipitates?

A

Cryofibrinogen precipitates have a cylindrical configuration in ultrastructural analysis within the lumina.

119
Q

What are the common complications associated with cryoglobulinemia?

A

Common complications include:

  1. Gangrene (5%)
  2. Septicemia (5%)
  3. Leg amputation (3.3%).
120
Q

What is the management strategy for essential cryofibrinogenemia?

A

Management strategies include:

  • Fibrinolytics and immunosuppressives
  • Oral stanozolol (2-4 mg twice daily)
  • Streptokinase (sometimes with streptodornase)
  • Colchicine (0.6 mg twice daily) with high-dose pentoxifylline (800 mg 3 times daily)
  • Prednisone (10-60 mg/d) with low-dose aspirin for nonsevere cases
  • Azathioprine (150 mg/d) or chlorambucil (10 mg/d) for severe cases.
121
Q

What is the significance of a cryocrit greater than 1% in cryoglobulinemia diagnosis?

A

A cryocrit greater than 1% is considered to be positive, indicating the presence of cryoglobulins in the serum.

122
Q

What is the most common cause of death in HCV patients with mixed cryoglobulinemia?

A

The most common cause of death in HCV patients with mixed cryoglobulinemia is related to liver disease.

123
Q

What is the relationship between mixed cryoglobulinemia and HCV?

A

The vast majority of mixed cryoglobulinemias occur in association with HCV infection.

124
Q

What is the treatment of choice for type I cryoglobulinemia?

A

The treatment of choice is typically immunosuppressive therapy.

125
Q

What is the preferred treatment for severe or refractory/relapsing type II and III cryoglobulinemia?

A

The preferred treatment is Rituximab in combination with corticosteroids and immunosuppressive therapy.

126
Q

What are the thromboembolic conditions associated with Type I cryoglobulinemia and cryofibrinogenemia?

A

Thromboembolic conditions include inherited or acquired hypercoagulable states, thrombocytopenic disorders, paroxysmal nocturnal hemoglobinuria, disseminated intravascular coagulation, atherosclerotic peripheral vascular disease, thromboangiitis obliterans, livedoid vasculopathy, and drug-induced conditions.

127
Q

What are inherited or acquired hypercoagulable states?

A

Conditions include protein C or S deficiency, antiphospholipid syndrome, and lupus anticoagulant.

128
Q

What thrombocytopenic disorders are associated with cryoglobulinemia?

A

Conditions include thrombotic thrombocytopenic purpura and idiopathic thrombocytopenic purpura.

129
Q

What is paroxysmal nocturnal hemoglobinuria?

A

It is a condition associated with cryoglobulinemia.

130
Q

What is disseminated intravascular coagulation?

A

It is a condition associated with cryoglobulinemia.

131
Q

What is atherosclerotic peripheral vascular disease?

A

It is a condition associated with cryoglobulinemia.

132
Q

What is thromboangiitis obliterans?

A

It is a condition associated with cryoglobulinemia.

133
Q

What is livedoid vasculopathy?

A

It is a condition associated with cryoglobulinemia.

134
Q

What drug-induced conditions are associated with cryoglobulinemia?

A

Examples include heparin and warfarin.

135
Q

What are atheroemboli?

A

Conditions include cholesterol emboli, septic emboli, and atrial myxoma.

136
Q

What causes hyperviscosity in cryoglobulinemia?

A

Causes include thrombocytosis (essential or secondary) and polyclonal or monoclonal gammopathies that are not cryoglobulins.

137
Q

What primary cutaneous disorders are associated with Type I cryoglobulinemia and cryofibrinogenemia?

A

Conditions include urticaria, livedo or livedoid vasculitis, neutrophilic dermatoses, lipodermatosclerosis, paniculitis, idiopathic perniosis (chilblains), calciphylaxis, hemoglobinopathies, and infections.

138
Q

What small- and medium-vessel vasculitis conditions are associated with Types II and III cryoglobulinemia?

A

Conditions include IgA vasculitis, ANCA-associated vasculitis, and rheumatic vasculitides.

139
Q

What are the thromboembolic conditions associated with Type I cryoglobulinemia and cryofibrinogenemia?

A

Conditions include inherited or acquired hypercoagulable states, thrombocytopenic disorders, and paroxysmal nocturnal hemoglobinuria.

140
Q

What is a common clinical presentation of conditions associated with small- and medium-vessel vasculitis?

A

Palpable purpura is a common presentation.

141
Q

What are some drug-induced conditions that can lead to cryoglobulinemia?

A

Conditions include those related to bacterial endocarditis and infections like Rickettsia and Neisseria meningitidis.

Fitzpatrick's Dermatology, 9th Edition (Chapter-based Decks) (48 decks)

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