Multiple myeloma

Dedicated to my Brother Sanjay Singh

Who left us for heaven on 22-11-2016 due to

Plasma cell Leukemia, a complication of Multiple Myeloma

Multiple Myeloma (Plasma Cell Myeloma)

Multiple Myeloma is a cancer of Plasma cells. Plasma cells are white blood cells that secrete large volumes of antibodies. Plasma cells originate in the bone marrow and are transported by the blood plasma and the lymphatic system. There is abnormal plasma cell production and a subsequent overabundance of monoclonal M protein which causes kidney problems and thickening of blood, also cause formation of mass in the bone marrow or soft tissue. When only one mass is present, it is referred as Plasmacytoma. When more than one mass is present, it is referred as Multiple Myeloma. Multiple Myeloma usually occurs in elderly and is more common in men.

Cause:

Ø  Unknown

Ø  Drinking alcohol and obesity are risk factors. Each increase of body mass index by 5 is supposed to increase the risk by 11%.

Ø  Familial predisposition to myeloma exists.

Survival:

Ø  Without treatment, survival is supposed to be around 7 months.

Ø  With current treatments, survival is usually 4 to 5 years.

Ø  Above mentioned survival periods are shocking for a newly diagnosed MM person but I would like to share my personal experience of the reality I believe the reason of such short life expectancy in my next note "Life Expectancy of Newly diagnosed Multiple Myeloma patient: My family's experience"

Symptoms:

1.      Bone pain: The diagnosis is incidental in 30% of cases, discovered during routine blood screening done for unrelated problems. 70% of patients have bone pain at presentation. Sudden pain, usually worse with movement and at night due to a broken bone in the spine, ribs, or elsewhere. Local bone damage and osteoporosis (general thinning of the bone)

2.      Anemia: Persistent tiredness and fatigue due to anemia. Anemia is because normal red blood cells are crowd by myeloma plasma cells.

3.      Kidney damage or failure: High Blood calcium level result in kidney damage, causing weight loss, nausea, thirst, muscle weakness, and mental confusion.

4.      Recurrent unexplained infections: Like pneumonia, sinus, or urinary infection

5.      Swelling over body, shortness of breath or evidence of heart or kidney failure.

6.      Bleeding: Bleeding resulting from thrombocytopenia. Bleeding from nose (Epistaxis) may be a presenting symptom.

 Lab test findings:

1.      Decrease Hb%, WBCs, Platelets

2.      Increased Bl. Calcium, Bl. Creatinine, Protein level in the blood and/or urine. A 24-hour urine collection for quantification of the Bence Jones protein (ie, lambda light chains), protein, and creatinine clearance is required.

3.      M-SPIKE: Presence of monoclonal protein in the blood and/or urine. The M-SPIKE is produced by the cancerous myeloma cells present in the bone marrow. In general, the amount of M-SPIKE reflects the amount of myeloma.

4.      Lytic bone lesions or osteoporosis. A conventional complete plain skeletal survey usually depicts lytic lesions.

5.      C-reactive protein (CRP) is useful for prognosis.

6.      Bone marrow aspirate and biopsy: To calculate the % of plasma cells,

7.      The Kappa/Lambda Ratio:

a.      When the level of either kappa or lambda is very high and the other chain is normal or low, then the ratio is abnormal and indicates that the myeloma is active.

b.      When the levels of both kappa and lambda light chains are increased, the ratio may be within the normal range, it generally indicates a disease other than myeloma, such as poor kidney function.

c.       When the kappa and lambda levels are both within the normal range but the ratio abnormal, then there may be a persistent low level of active myeloma with excess production of the abnormal light chains.

d.      A normal kappa/lambda ratio after treatment is a particularly good remission and is termed a stringent complete response. Normalization of the kappa/lambda ratio correlates with possible longer remissions.

 MGUS- monoclonal gammopathy of undetermined significance: This is predominantly a benign condition. The risk of developing active myeloma is very low: 1%/year. Thus, after 20 years, 80% of patients have not developed active myeloma. MGUS Diagnostic Criteria: All Three Required:

Ø  Serum monoclonal protein and/or urine monoclonal protein level low.

Ø  Monoclonal bone marrow plasma cells < 10%. Normal Serum Calcium, Hemoglobin and Serum Creatinine level.

Ø  No bone lesions on full skeletal X-ray survey and/or other imaging if performed.

Smoldering myeloma (low risk): Very similar to MGUS. Approximately 50% of patients will develop myeloma within 18-24 months. Smoldering or Indolent Myeloma Diagnostic Criteria: All Three Required:

Ø  Monoclonal protein present in the serum and/or urine

Ø  Monoclonal plasma cells present in the bone marrow and/or a tissue biopsy.

Ø  Not meeting criteria for MGUS, multiple myeloma, or solitary plasmacytoma of bone or soft tissue.

Plasma Cell Leukemia: A dreaded complication of Multiple Myeloma. The survival rate of these patients with present available treatment modalities are very poor.

Treatment:

1.      Currently No Cure for MM.

2.      Autologous stem cell transplantation, radiation and surgical care in certain cases, have helped to lessen the occurrence and severity of adverse effects of this disease.

3.      Chemotherapy and immunosuppression: Several drug therapies are valuable in the treatment of symptomatic MM.

a.      Thalidomide, either as a single agent or in combination with steroids.

b.      Lenalidomide plus dexamethasone

c.       Bortezomib plus melphalan

d.      VAD (vincristine, doxorubicin and dexamethasone)

e.      Melphalan plus prednisone

4.      Adjunctive therapy may also include:

a.      Erythropoietin

b.      Surgical intervention

c.       Plasmapheresis

5.      Autologous Stem Cell Transplant: The first step before starting therapy in MM is to determine whether a patient is a candidate for an autologous stem cell transplant. Eligibility depends primarily on the patient’s age and comorbidities. Typically an age of 65 years is used as a cut-off point for transplant eligibility. Using the patient’s own bone marrow or peripheral blood stem cells facilitates more intense therapy for MM. After harvesting the stem cells from the patient, physicians can use otherwise lethal doses of total body irradiation and chemotherapy and then “rescue” the patient by reinfusing the harvested cells.

6.      Physical Activity: Patients should be encouraged to be physically active to the extent appropriate for their individual bone status. Physical activity may help maintain bone strength.

7.      Black Cumin seed oil: Thymoquinone, an extract of black cumin seed oil is shown to be effective suppressor of tumour cell survival, proliferation and angiogenesis in patient of Multiple Myeloma in one study.

Warning: The purpose of this article is to create awareness for the disease mentioned above. The reader of this article should exercise all precautions before following any information provided above and it is advised that you consult your own physician or other medical professional. The responsibility lies solely with the reader and not with the site or the writer.