Cardio Renal Amyloidosis (Non-Secretory Myeloma)

Josephine Angel

Seniro Staff Nurse, Kauvery Heartcity, Trichy, India

Background Definition

Multiple myeloma is the growth of abnormal cells in the bone marrow.

Amyloidosis is the name for a group of rare and serious conditions caused by a buildup of an abnormal protein called amyloid in the organs and tissues throughout the body

The buildup of amyloid protein can occur in the heart, kidneys, liver and other organs.  The abnormal protein is produced in the bone marrow.

Main Cause

This is caused by fragments of amyloid, a protein, which affects the organ it infiltrates. It complicates chronic diseases such as

  1. Rheumatic disease (Rheumatic Arthritis)
  2. Chronic inflammatory bowed disease
  3. Tuberculosis or empyema

Types

  1. AL (Primary Amyloidosis)- Amyloid & light chain
  2. AA (Secondary amyloidosis) amyloid & serum a protein
  3. Familial ATTR amyloidosis (Amyloid transport protein transthyretin)

Classification

  1. Systemic
  2. Hereditary
  3. Ocular
  4. Central nervous system
  5. Localized etiology

Amyloidosis Symptoms

  1. Urine changes and swollen legs
  2. Unintentional significant weight loss
  3. Severe fatigue
  4. Shortness of breath
  5. Numbness, tingling sensation in hands and feet
  6. Weakness or pain in hands or feet
  7. Diarrhea or Constipation
  8. An enlarged tongue
  9. Skin changes

Non-Secretory Multiple Myeloma Definition

Non-secretory myeloma patients can be divided into several groups.  The true non secretory myeloma should be considered only in patients whose tumors have a defect in immunoglobulin synthesis, resulting in no measureable protein in the blood or urine, although they still have a significant plasma cell burden in the bone marrow and evidence of end organ damage.

Case Presentation

A 52 years old male presented with complaints of breathlessness and pedal edema in May’22. He was evaluated by cardiologist and found to have diastolic heart failure with mid range ejection fraction   In view of normocytic anaemia, he was referred here for further management.

Signs and Symptoms

  1. Breathlesness
  2. Pedal edema
  3. Fatigue

Investigations

  1. Raised Uric acid
  2. Raised LDH
  3. Serum protein electrophoresis – hypogamma globulinemia
  4. Immunofixation – no monoclonal gammapathy
  5. Fluorescence in situ hybridization (Fish ) for TP53 – Negative
  6. Cardiac MRI – Suggetive of infiltrative disease (amyloidosis)
  7. Urine protein – 810mg/24hr
  8. Trop I (Quantitative)- 0.06 – 07/0/2023
  9. -0.13mg/ml – 13/01/2023
  10. Serum Globulin – 2.6g/dl
  11. Serum Albumin – 3.04g/dl
  12. Urine Albumin – (++)
  13. Urine Acetone – Negative
  14. NTpro BNP – 2514 pd/ml
  15. ECG-report attached
  16. ECHO- report attached
  17. USG Abdomen : Report attached
  18. CAG : report attached
  19. Bone marrow aspiration with trephine:
  20. Biopsy – Hypocellular marrow with no incerase in plasma cells or evidence of amyloid deposit

ECG

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ECHO Report

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USG Abdomen

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CAG Report: Normal

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Bone Marrow Report

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Lab Report

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Urine Report

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Blood Culture Report

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Treatment

  1. He was started on CyborD(Cyclophosphamide + Bortezomib + Dexamethasone) regimen (chemotherapy) for 7 cycles (21/05/22)
  2. Peripheral blood autologous Stem Cell transplantation on 18/12/2022

Nursing Management

  1. A head-to-toe assessment was conducted to evaluate health status responses to medications and other therapies and pain or discomfort, which may be affecting the patients’ activities of daily living.
  2. Assessment of emotional support is also essential
  3. The assessment includes an evaluation for protein in the urine, cardiopulmonary involvement, nerve damage, gastrointestinal function, including malabsorption and cutaneous lesions including bruising.

Recovery

Patient became stable with the efficient care of nurses and went home happily with good prognosis.

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Ms. Josephine Angel

Senior Staff Nurse

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