Patient: [Patient Name] MRN: [Medical Record Number] Date: [Date of Visit]
Chief Complaint:
Document the patient’s main concerns related to AL amyloidosis. These can vary depending on affected organs and may include:
Fatigue (common)
Weakness
Weight loss
Shortness of breath (if lungs involved)
Diarrhea (if gastrointestinal tract involved)
Peripheral neuropathy (numbness, tingling in hands and feet)
Swelling of the tongue or other tissues (less common)
History of Present Illness:
Age at onset of symptoms
Duration and progression of symptoms
Specific symptoms related to the suspected organ involvement
Prior hospitalizations (if any)
Past Medical History:
Underlying medical conditions (e.g., monoclonal gammopathy of undetermined significance (MGUS), multiple myeloma)
Prior surgeries or procedures
History of infections (potential triggers)
Family History:
Family history of blood cancers (uncommon)
Social History:
Current medications and supplements
Smoking history (may worsen some presentations)
Physical Examination:
Vital signs (may be normal or show signs of organ dysfunction)
General examination: signs of wasting (muscle loss) and fatigue
Cardiovascular exam (assess for fluid overload or heart failure)
Pulmonary exam (assess for lung involvement)
Abdominal exam (assess for organomegaly, ascites)
Peripheral neuropathy examination (assess for sensory or motor deficits)
Laboratory Tests:
Serum protein electrophoresis (SPEP) and immunofixation electrophoresis (IFE):
Identify presence of an abnormal monoclonal protein (M-protein)
Serum free light chain assay:
Quantify the involved light chain type (kappa or lambda)
Urine protein electrophoresis (UPEP) and immunofixation electrophoresis (IFE):
Detect presence of M-protein in the urine (important for diagnosis)
Complete blood count (CBC):
May show anemia or low white blood cell count
Kidney function tests:
May be abnormal if kidneys are involved
Liver function tests:
May be abnormal if liver is involved
Other tests may be done depending on suspected organ involvement (e.g., cardiac biomarkers for heart involvement)
Imaging Studies:
Imaging studies may be used to assess organ involvement and guide biopsy sites.
Chest X-ray: Assess for lung involvement.
Echocardiogram: Assess for heart function and damage.
CT scan/MRI (depending on suspected organ involvement): Evaluate kidneys, liver, other organs for signs of amyloidosis.
Bone marrow biopsy:
May be performed to identify underlying plasma cell dyscrasia (e.g., MGUS, multiple myeloma).
Tissue Biopsy:
Obtaining tissue samples from affected organs (e.g., kidney, heart, nerve) is crucial for definitive diagnosis.
Congo red stain and electron microscopy confirm the presence of amyloid deposits.
Diagnosis:
Immunoglobulin light chain amyloidosis (AL amyloidosis):
Based on clinical presentation, laboratory findings (M-protein, positive urine light chains), and confirmation by tissue biopsy with Congo red stain and electron microscopy.
Specify the organs involved (e.g., AL amyloidosis with cardiac involvement, renal involvement).
Staging (optional):
Some staging systems categorize AL amyloidosis based on the severity of organ dysfunction.
Treatment Plan:
Treatment aims to suppress the production of abnormal light chains and prevent further amyloid deposition.
The specific plan depends on the presence of underlying plasma cell dyscrasia (MGUS, multiple myeloma) and the severity of organ involvement. May include:
Chemotherapy: To target and destroy abnormal plasma cells.
Proteasome inhibitors: Newer medications that block protein degradation pathways in plasma cells.
**Immunomodulatory drugs (IMiDs