Cryoglobulinemia is a medical condition in which blood contains large amounts of cryoglobulins-proteins that become insoluble at reduced temperatures.
Cryoglobulinemia can be associated with various diseases such as multiple myeloma and hepatitis C infection.
Age/Sex prevalance of cryoglobulinemia
The female to male ration of occurrence is 3:1
The most common age group to be affected by cryoglobulinemia is between 42-52 years of age.
No racial predominance is seen.
Cryoglobulinemia is classifically grouped into three types according to brouet classification and the three types are as follows
• Type І cryoglobulinemia is most commonly encountered in patients with a plasma cell dyscrasia such as
• Type ΙΙ and type ІΙІ of cryoglobulinemia are strongly associated with infection by the hepatitis C virus.
• Type ΙІΙ of cryoglobulinemia is associated with autoimmune diseases such as given below
Systemic lupus erythematous
Disease associations’ variable based on type of cryoglobulinemia are given as under
• Type І cryoglobulinemia is seen in lymphoproliferative disorders example multiple myeloma, walsenstrom macroglobulinemia.
• Type ΙІ and ΙІΙ are observed in chronic inflammatory diseases such as chronic liver disease
Coexistent connective- tissue diseases
Mixed cryoglobulinemia is rarely associated with lymphoproliferative disorders.
Infections in which cryoglobulinemia is seen
• Viral infections are as follows
Hepatitis A, B, C
• Bacterial infections
• SLE, rheumatoid arthritis, sjogren syndrome
• Vasculitis- polyarteritis nodosa, Henoch-schonlein purpura
• Livedoid vasculitis
• Cold-induced urticaria
• Hyperkeratotic spicules in areas exposed to cold
• Scarring of tip of nose, pinnae, fingertips and toes
• Nailfold capillary abnormalities
• Palpable pupura
• Ischemic necrosis
• Pleural effusion
• Abdominal pain
• Hepatomegaly or signs of cirrhosis’
• Membranoproliferative glomerulonephritis
• Intraluminal cryoglobulin deposition
• Nephrotic-range proteinuria with resultant edema
• Frank arthritis and progressive joint deformity
Nervous system manifestations
• Sensorimotor neuropathy
• Visual disturbances
• CNS involvement
• Evaluation of serum cryoglobulins
• Complete blood cell count
• Serum chemistry
• Liver function studies
• Rheumatoid factor
• Antinuclear antibody
• Erythrocyte sedimentation rate
• Complete evaluation
Other studies to be done are as follows
• Consider serum protein electrophoresis
• Urine protein electrophoresis
• Quantitative immunoglobulin
Imaging studies to be done are as follows
• A chest radiograph
• CT imaging
• Transesophageal echocardiography
Tissue biopsy required for diagnosis when patients are evaluated with vasculitis.
Electromyography and nerve conduction studies are also done
Further diagnostic procedures to be done are as follows
• Bone marrow biopsy
• Liver or kidney biopsy.
Cryoglobulinemia is treated with plasmapheresis.
Severe cryoglobulinemia which involves vital organs or large areas of skin is treated by corticosteroids and other medicines that calm the immune system.
• Stroke, seizure or coma
• Congestive heart failure
• Respiratory distress
• Gastrointestinal hemorrhage
• Acute renal failure
• Severe cutaneous necrosis or gangrene
The prognosis in these patients depends on the presence of underlying diseases.
• Chronic lymphocytic leukemia
• Churg-Strauss syndrome
• Giant cell arteritis
• Glomerulonephritis, acute
• Diffuse proliferative glomeruonephritis
• Goodpasture syndrome
• Hemolytic-uremic syndrome
• Viral hepatitis
• Non-hodgkin lymphoma
• Serum sickness
• Systemic lupus erythematous
• Antiphospholipid syndrome
• Hepatitis A
• Hepatitis B
• Hepatitis C