Cryoglobulinemia

What is cryoglobulinemia?

Cryoglobulinemia is a medical condition in which blood contains large amounts of cryoglobulins-proteins that become insoluble at reduced temperatures.

What are the risk factors of Cryoglobulinemia?

• Blindness 

• Stroke, seizure or coma
• Pericarditis
• Congestive heart failure
• Respiratory distress
• Gastrointestinal hemorrhage
• Acute renal failure
• Severe cutaneous necrosis or gangrene

Is Cryoglobulinemia a cancer?

Cryoglobulinemia is generally broken down in three subtypes. Type I cryoglobulinemia is usually associated with an underlying disorder, specifically certain types of cancer. Type II and type III cryoglobulinemia also called as mixed cryoglobulinemia.

What is the Classification of Cryoglobulinemia?

Cryoglobulinemia is classifiably 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
-Multiple myelomas

-Waldenstrom macroglobulinemia

• 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 erythematosus

-Rheumatoid arthritis

-Disease associations’ variable based on type of cryoglobulinemia are given as under

• Type І cryoglobulinemia is seen in lymphoproliferative disorders example multiple myeloma, Waldenstrom macroglobulinemia.

• Type ΙІ and ΙІΙ are observed in chronic inflammatory diseases such as
-chronic liver

-Disease Infections

-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

-HIV
-Cytomegalovirus
-Adenovirus
• Bacterial infections

-Syphilis
-Lyme disease

-Leprosy
-Q-fever
-Brucellosis
-Endocarditis
-Streptococcal infections

• Fungal

-Coccidioidomycosis

• Parasitic

-Malaria
-Toxoplasmosis
-Autoimmune diseases

•SLE, rheumatoid arthritis, Sjogren’s syndrome

• Vasculitis- polyarteritis nodosa, Henoch-schonlein purpura

What are the signs and symptoms of cryoglobulinemia

Skin manifestation

• Livedoid vasculitis

• Cold-induced urticaria

• Hyperkeratotic spicules in areas exposed to cold

• Scarring of nose tip, pinnae, fingertips, and toes

• Acrocyanosis

• Nailfold capillary abnormalities

• Palpable purpura

• Ischemic necrosis

Pulmonary manifestation

• Dyspnea

• A cough

• Pleurisy

• Pleural effusion

• Bronchiectasis

Gastrointestinal manifestations

• Abdominal pain

• Hemorrhage

• Hepatomegaly or signs of cirrhosis’

• Splenomegaly

Renal manifestations

• Membranoproliferative glomerulonephritis

• Intraluminal cryoglobulin deposition

• Hypertension

• Nephrotic-range proteinuria with resultant edema

Joint manifestation

• Arthralgias

• Frank arthritis and progressive joint deformity

Nervous system manifestations

• Sensorimotor neuropathy

• Visual disturbances

• CNS involvement

Can cryoglobulinemia be treated?

In severe cases, treatment can help keep flares to a minimum and reduce the overall severity of the symptoms. Hepatitis C, blood cancers, and underlying causes of cryoglobulinemia will require treatment and prognosis will depends upon the person immunity.

Cryoglobulinemia is treated with plasmapheresis.
Severe cryoglobulinemia which involves vital organs or large areas of skin is treated with corticosteroids and other medicines that calm the immune system.

What are the investigations for cryoglobulinemia?

• Evaluation of serum cryoglobulins

• Urine analysis

• 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

• Serum viscosity

Imaging studies to be done are as follows

• A chest radiograph

• CT imaging

• Transesophageal echocardiography

• Angiography

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.