Paraproteinameias Flashcards
What is polyneuropathy a part of?
POEMS syndrome
POEMS syndrome includes Polyneuropathy, endocrinopathy, organomegaly, monoclonal gammopathy, and skin changes.
What causes organomegaly in POEMS syndrome?
Deposition of plasma cells in the organs
What causes endocrinopathy in POEMS syndrome?
Deposition of amyloid in the endocrine organs
What are the features of neuropathy?
Tingling sensation and numbness of the limbs
Occasionally, paralysis of the limbs, bladder, or rectal dysfunction may occur.
Which investigations were requested by the attending physician?
FBC, ESR, EUC, LFT
What could be the cause of easy fatigability in the patient?
Possible anemia, underlying disease, or other systemic issues
The exact cause would depend on further investigations.
What type of anemia did the woman have based on FBC results?
Microcytic anemia
Based on Hct of 26%, MCV of 86fL, and other parameters.
What is plasma cell leukemia?
Rare and aggressive form of multiple myeloma
What percentage of WBC must be plasma cells to diagnose plasma cell leukemia?
Plasma cells >20% of total WBC or >2000 plasma cells/µL
What factors influence the prognosis of plasma cell leukemia?
Stage of disease, cytogenetics, age
What is the median survival for progressive plasma cell leukemia?
3-4 years
What is the first-line chemotherapy for managing plasma cell myeloma?
Melphalan with Prednisone (MP)
What is rouleaux formation?
Stacking of red blood cells resembling a stack of coins
Which conditions can cause rouleaux formation? (Choose all that apply)
- Infections/inflammatory disorders
- Multiple myeloma
- Cancers
What are the expected findings in serum protein electrophoresis for multiple myeloma?
Presence of a monoclonal band
Using the International Myeloma Working Group criteria, which of the following is not used to diagnose multiple myeloma?
CD 38 positivity
Which stage is indicated if serum β2-microglobulin is <3.5 mg/L and serum albumin is ≥3.5 g/dL?
Stage I
List the major differences between MGUS, smoldering myeloma, and multiple myeloma.
- MGUS: Serum paraprotein <30 g/L, clonal plasma cells <10%
- Smoldering myeloma: Serum paraprotein >30 g/L, clonal plasma cells >10%
- Multiple myeloma: Clonal plasma cells >10%, evidence of organ damage
What does Bence Jones protein consist of?
Free heavy and light chains
True or False: Anaemia in multiple myeloma can be due to renal failure.
True
Which of the following is an osteoclast activating factor for multiple myeloma?
IL-6
Which chromosomal abnormalities are associated with poor prognosis in multiple myeloma?
T(4;14) and T(14;16)
What supportive treatment is recommended for pain management in multiple myeloma?
Analgesics, local radiotherapy, spinal support corset
What is the diagnostic criterion for symptomatic multiple myeloma?
Clonal plasma cells in bone marrow >10%, monoclonal protein in serum or urine, evidence of end-organ damage
What is the most common type of myeloma?
IgG myeloma
What is the feature that may not be commonly associated with multiple myeloma?
Gallstones
True or False: Smoldering myeloma is considered an aggressive disease.
False
What is the heat precipitation test used to identify?
Bence Jones protein
What is the expected serum calcium level in multiple myeloma?
Elevated serum calcium
What is a common immunophenotypic feature of multiple myeloma?
CD 38
What is the role of bisphosphonates in multiple myeloma management?
To manage bone disease/hypercalcemia
What is plasma cell myeloma (multiple myeloma)?
A malignant, multifocal plasma cell neoplasm of the bone marrow associated with an M-protein in serum and/or urine.
What is the most common monoclonal gammopathy?
Multiple myeloma.
What are the common presentations of multiple myeloma?
Multiple tumour masses throughout the skeletal system.
What usually precedes multiple myeloma?
Asymptomatic plasma cell dyscrasia - MGUS and smouldering myeloma.
What are some diseases associated with paraproteinaemias?
- Multiple myeloma
- Smoldering myeloma
- Monoclonal gammopathy of undetermined significance (MGUS)
- Plasma cell Leukemia
- Waldenstrom’s macroglobulinemia
- Heavy chain disease
- Monoclonal gammopathy of renal significance (MGRS)
- Cold agglutinin disease (CAD)
- TEMPI syndrome
- AESOP syndrome
What causes renal failure in plasma cell disorders?
Precipitated L chains induce interstitial nephritis and hypercalciuria may cause obstructive nephropathy.
What is a paraprotein?
Immunoglobulin produced from a single clone of plasma cells, also referred to as M-protein.
What is paraproteinaemia?
Presence of a paraprotein in plasma in excessive amounts.
What are the categories of paraproteinaemias based on monoclonal protein?
- Light chains only (Bence Jones protein)
- Heavy chains only (heavy chain disease)
- Whole immunoglobulins (M-protein)
What is a Mott cell?
A plasma cell variant with a classic ‘bunch of grapes’ appearance due to Ig inclusions (Russell bodies).
What is the primary cytokine involved in the pathophysiology of multiple myeloma?
Interleukin 6 (IL-6).
What are the clinical features of bone pain in multiple myeloma?
Direct effect of osteoclast activating factors and cytokines leading to bone demineralization.
What are common symptoms of renal failure in multiple myeloma?
- Polyuria
- Polydipsia
- Anorexia
- Vomiting
- Constipation
What are the features of anemia in multiple myeloma?
- Lethargy
- Weakness
- Pallor
- Tachycardia
What chromosomal abnormality is commonly associated with multiple myeloma?
Abnormalities involving chromosome 14, such as t(11;14), t(4;14), and t(14;16).
What is the epidemiology of multiple myeloma in terms of race?
The incidence in blacks is almost twice that in Caucasians.
What is the median age at diagnosis for multiple myeloma?
66 years.
True or False: Multiple myeloma has a slight male predominance.
True.
Fill in the blank: The abnormal plasma cell variant known as _______ has cytoplasm resembling a red flame.
Flame Cell
What is TEMPI syndrome?
A syndrome characterized by telangiectasia, elevated erythropoietin, erythrocytosis, monoclonal gammopathy, perinephric fluid collection, and intrapulmonary shunting.
