Conditions related to multiple myeloma

There are several conditions related to multiple myeloma. Some conditions may occur in people diagnosed with multiple myeloma. Other conditions don’t develop in people with multiple myeloma but these conditions have some similar features as multiple myeloma.

Osteosclerotic myeloma (POEMS syndrome)

Osteosclerotic myeloma (POEMS syndrome) is a rare disorder that affects multiple systems of the body. The name POEMS syndrome comes from the most common features of the disease:

Peripheral neuropathy causes weakness, loss of sensation, pins and needles or burning pain in the hands and feet. It is usually the worst symptom of POEMS syndrome.

Organomegaly refers to organ enlargement. People with POEMS syndrome often have larger than normal liver, spleen and lymph nodes.

Endocrinopathy is a term used to describe the endocrine system when it isn’t working right. The endocrine system is the group of hormone-producing glands and tissues that help regulate growth, sexual development and other body functions.

M-proteins are found in the blood and urine. There may also be plasma cell tumours, called plasmacytomas.

Skin changes might include skin darkening, thickening or tightening. Other changes that may occur include hair growth on the face, limbs and chest, swelling (edema) of the legs and feet, and whitening of the nails.

Treatments

Treatment options for osteosclerotic myeloma include:

  • radiation therapy
  • chemotherapy
  • stem cell transplant
  • targeted therapy
  • hormone replacement therapy (for those whose endocrine organs are affected)

Waldenstrom macroglobulinemia

People with Waldenstrom macroglobulinemia make too much immunoglobulin M (IgM). A high amount of IgM in the blood will cause the blood to thicken, leading to a condition called hyperviscosity syndrome. Hyperviscosity causes problems with the circulation of the blood through the body. Signs and symptoms of hyperviscosity syndrome includes headaches and feeling dizzy, weak or tired.

Treatments

Treatment options for Waldenstrom macroglobulinemia include:

  • watchful waiting – only used in those who don’t have symptoms
  • plasmapheresis – uses a machine to remove plasma and IgM from the blood when it is too thick
  • chemotherapy
  • targeted therapy
  • stem cell transplant
  • surgery to remove the spleen (splenectomy) if the spleen is enlarged

Light chain amyloidosis

Amyloid refers to a protein or part of a protein that forms abnormal clumps in the body. In light chain amyloidosis, the light chains of an immunoglobulin build up in tissues of the body (called amyloid deposits). Amyloid can build up in any tissue and interfere with its function. Light chain amyloidosis affects about 10%–15% of people with multiple myeloma but it can also develop in people who don’t have the disease. It may also be associated with other B-cell cancers, such as lymphoma and chronic lymphocytic leukemia (CLL).

Amyloid is sometimes seen on a bone marrow biopsy sample, but a doctor may need to biopsy other tissues, such as the fat on the abdomen (belly), to look for amyloid. In rare cases, a doctor will biopsy the heart or kidneys to look for amyloid if a person is having heart or kidney problems.

Treatments

Treatment for amyloidosis is similar to treatment for multiple myeloma. It targets the abnormal plasma cells. Treatment options include:

  • chemotherapy
  • radiation therapy
  • targeted therapy
  • stem cell transplant

Plasma cell leukemia

Plasma cell leukemia (PCL) is a rare, aggressive condition where large numbers of plasma cells circulate in the blood. The circulating plasma cells may be the first evidence of disease (called primary PCL) or may develop in the later stages of multiple myeloma (called secondary PCL). Most cases of PCL are primary PCL. PCL is more aggressive than multiple myeloma.

Treatments

Treatment options for PCL include:

  • chemotherapy
  • stem cell transplant

Heavy chain disease

Heavy chain disease is a rare disease of B cells (also called B lymphocytes). In heavy chain disease, plasma cells make incomplete immunoglobulin molecules that only have the heavy chain part of the immunoglobulin. There are 3 types of heavy chain disease, which are classified according to the type of heavy chain.

Find out more about immunoglobulins.

IgA heavy chain disease

Heavy chains of IgA are found in IgA heavy chain disease (also called alpha heavy chain disease). IgA is the most common type of heavy chain disease, and it affects the gastrointestinal tract. Experts believe it is caused by long-term stimulation of the immune system by bacteria. Treatment options for IgA heavy chain disease include chemotherapy and antibiotics with or without corticosteroids.

IgG heavy chain disease

Heavy chains of IgG are found in IgG heavy chain disease. IgG heavy chain disease is a type of lymphoproliferative disorder where lymphocytes are made in high amounts. Some people have a mild form of the disease while others have an aggressive form. Treatment options for IgG heavy chain disease include watchful waiting for those with a mild form of the disease, radiation therapy and chemotherapy.

IgM heavy chain disease

IgM heavy chain disease sometimes occurs in people with chronic lymphocytic leukemia (CLL). Some people have a mild form of the disease while others have an aggressive form. Treatment options for IgM heavy chain disease include watchful waiting and chemotherapy.

Expert review and references

  • Alberta Health Services. Multiple Myeloma Clinical Practice Guideline LYHE-003. Alberta Health Services; 2015.
  • Baz R, Huseein MA . Uncommon presentations of plasma cell dyscrasias. Raghavan D, Blanke CD, Honson DH, et al (eds.). Textbook of Uncommon Cancer. 4th ed. Wiley Blackwell; 2012: 45: 627-636.
  • Berenson JR . Heavy chain diseases. Beers, M. H., & Berkow, R., (Eds.). Merck Manual Professional Edition. 2013: http://www.merckmanuals.com/professional/hematology-and-oncology/plasma-cell-disorders/heavy-chain-diseases.
  • Blade J, Rosinol L . Complications and special presentations of plasma cell myeloma. Wiernik PH, Goldman JM, Dutcher JP, Kyle RA (eds.). Neoplastic Diseases of the Blood. 5th ed. Springer; 2013: 34: 665-680.
  • Dispenzieri A, Dingli D, Gertz MA . POEMS syndrome, cryoglobulinemia, and heavy-chain disease. Greer JP, Arber DA, Glader B, List AF, Means RT Jr, Paraskevas F, Rodgers GM, Foerster J, (eds.). Wintrobe's Clinical Hematology. 13th ed. Lippincott Williams & Wilkins; 2014: 101: 2141-2158.
  • Dispenzieri A, Lacy MQ, Kumar S . Multiple myeloma. Greer JP, Arber DA, Glader B, List AF, Means RT Jr, Paraskevas F, Rodgers GM, Foerster J, (eds.). Wintrobe's Clinical Hematology. 13th ed. Lippincott Williams & Wilkins; 2014: 98: 2046-2097.
  • Kastritis E, Dimopoulos MA . Waldenstrom's macroglobulinemia. Wiernik PH, Goldman JM, Dutcher JP, Kyle RA (eds.). Neoplastic Diseases of the Blood. 5th ed. Springer; 2013: 101: 2141-2158.
  • Myeloma Canada. Multiple Myeloma Patient Handbook. Third ed. Kirkland, QC: Myeloma Canada; 2014.
  • The Leukemia & Lymphoma Society. Myeloma. Revised ed. White Plains, NY: The Leukemia & Lymphoma Society; 2013: http://www.llscanada.org/content/nationalcontent/resourcecenter/freeeducationmaterials/myeloma/pdf/myeloma.pdf.

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