Waldenström's macroglobulinemia

In essence Waldenström's macroglobulinemia is a malignant mutation of B-cells,and its proliferation and differentiation into lymphoplasmocytic cells producing monoclonal immunoglobulin IgM. It is 10 times less common than multiple myeloma. It is one of the diseases called monoclonal gammopathy.

The clinical picture

 * Bone marrow infiltration by lymphoplasmocytic cells with suppression of normal hematopoiesis and circulatory deficiency and / or IgM monoclonal immunoglobulin, which increases plasma viscosity (large molecule),
 * chronic fatigue, feeling weak and inefficient,
 * later bleeding from the nose, gums ( due to hyperviscocity)
 * osteolysis (x in contrast to multiple myeloma sparse bone pain),
 * chronic headache (increased plasma volume + increased intracranial pressure ),
 * heart failure, shortness of breath , congestive heart failure; later visual difficulties (hyperviscosity + increased plasma volume),
 * slowly progressing symmetric peripheral neuropathy (caused by monoclonal immunoglobulin as in all gammopathy),
 * monoclonal immunoglobulin type IgM can cause other symptoms, have the property of cryoglobulin, or. cold agglutinins.

Diagnosis

 * ↑ ESR (around 100 / h) → we examine Ig quantitatively + examine the presence of monoclonal Ig,
 * ↑ total IgM / presence of monoclonal IgM / cytopenia → trepanobiopsy ,
 * the value of β2 – microglobulin correlates with the size of the tumor mass + its aggressiveness,
 * higher plasma viscosities.

Differential diagnosis

 * MGUS (Monoclonal gammopathy of undetermined significance), IgM multiple myeloma, splenic lymphoma, hairy cell leukemia , CLL, etc.

Therapy

 * Less aggressive disease → treatment indicated only with signs of damage to the body,
 * monotherapy / combined cytostatic treatment (fludarabine + cyclophosphamide) supplemented or o anti-CD20 antibodies (rituximab; lymphoplasmocyte cells, in contrast to myeloma cells, express the CD20 antigen ),
 * a series of therapeutic plasmapheresis is necessary for alleviation of signs of hyperviscosity syndrome.

Prognosis

 * Its course similar to multiple myeloma (remission + relapses),
 * bad in resistance to treatment / transformation into a more aggressive type of disease,
 * the average survival is 5 years.

Source

 * ws:Waldenströmova makroglobulinemie

Related articles and external links

 * Multiple myeloma
 * IgM
 * Waldenström's macroglobulinemia (WIKIPEDIA)