Paraproteinameias

Question 1

What is polyneuropathy a part of?

Answer 1

POEMS syndrome

POEMS syndrome includes Polyneuropathy, endocrinopathy, organomegaly, monoclonal gammopathy, and skin changes.

Question 2

What causes organomegaly in POEMS syndrome?

Answer 2

Deposition of plasma cells in the organs

Question 3

What causes endocrinopathy in POEMS syndrome?

Answer 3

Deposition of amyloid in the endocrine organs

Question 4

What are the features of neuropathy?

Answer 4

Tingling sensation and numbness of the limbs

Occasionally, paralysis of the limbs, bladder, or rectal dysfunction may occur.

Question 5

Which investigations were requested by the attending physician?

Answer 5

FBC, ESR, EUC, LFT

Question 6

What could be the cause of easy fatigability in the patient?

Answer 6

Possible anemia, underlying disease, or other systemic issues

The exact cause would depend on further investigations.

Question 7

What type of anemia did the woman have based on FBC results?

Answer 7

Microcytic anemia

Based on Hct of 26%, MCV of 86fL, and other parameters.

Question 8

What is plasma cell leukemia?

Answer 8

Rare and aggressive form of multiple myeloma

Question 9

What percentage of WBC must be plasma cells to diagnose plasma cell leukemia?

Answer 9

Plasma cells >20% of total WBC or >2000 plasma cells/µL

Question 10

What factors influence the prognosis of plasma cell leukemia?

Answer 10

Stage of disease, cytogenetics, age

Question 11

What is the median survival for progressive plasma cell leukemia?

Answer 11

3-4 years

Question 12

What is the first-line chemotherapy for managing plasma cell myeloma?

Answer 12

Melphalan with Prednisone (MP)

Question 13

What is rouleaux formation?

Answer 13

Stacking of red blood cells resembling a stack of coins

Question 14

Which conditions can cause rouleaux formation? (Choose all that apply)

Answer 14

• Infections/inflammatory disorders
• Multiple myeloma
• Cancers

Question 15

What are the expected findings in serum protein electrophoresis for multiple myeloma?

Answer 15

Presence of a monoclonal band

Question 16

Using the International Myeloma Working Group criteria, which of the following is not used to diagnose multiple myeloma?

Answer 16

CD 38 positivity

Question 17

Which stage is indicated if serum β2-microglobulin is <3.5 mg/L and serum albumin is ≥3.5 g/dL?

Answer 17

Stage I

Question 18

List the major differences between MGUS, smoldering myeloma, and multiple myeloma.

Answer 18

• 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

Question 19

What does Bence Jones protein consist of?

Answer 19

Free heavy and light chains

Question 20

True or False: Anaemia in multiple myeloma can be due to renal failure.

Answer 20

True

Question 21

Which of the following is an osteoclast activating factor for multiple myeloma?

Answer 21

IL-6

Question 22

Which chromosomal abnormalities are associated with poor prognosis in multiple myeloma?

Answer 22

T(4;14) and T(14;16)

Question 23

What supportive treatment is recommended for pain management in multiple myeloma?

Answer 23

Analgesics, local radiotherapy, spinal support corset

Question 24

What is the diagnostic criterion for symptomatic multiple myeloma?

Answer 24

Clonal plasma cells in bone marrow >10%, monoclonal protein in serum or urine, evidence of end-organ damage

Question 25

What is the most common type of myeloma?

Answer 25

IgG myeloma

Question 26

What is the feature that may not be commonly associated with multiple myeloma?

Answer 26

Gallstones

Question 27

True or False: Smoldering myeloma is considered an aggressive disease.

Answer 27

False

Question 28

What is the heat precipitation test used to identify?

Answer 28

Bence Jones protein

Question 29

What is the expected serum calcium level in multiple myeloma?

Answer 29

Elevated serum calcium

Question 30

What is a common immunophenotypic feature of multiple myeloma?

Answer 30

CD 38

Question 31

What is the role of bisphosphonates in multiple myeloma management?

Answer 31

To manage bone disease/hypercalcemia

Question 32

What is plasma cell myeloma (multiple myeloma)?

Answer 32

A malignant, multifocal plasma cell neoplasm of the bone marrow associated with an M-protein in serum and/or urine.

Question 33

What is the most common monoclonal gammopathy?

Answer 33

Multiple myeloma.

Question 34

What are the common presentations of multiple myeloma?

Answer 34

Multiple tumour masses throughout the skeletal system.

Question 35

What usually precedes multiple myeloma?

Answer 35

Asymptomatic plasma cell dyscrasia - MGUS and smouldering myeloma.

Question 36

What are some diseases associated with paraproteinaemias?

Answer 36

* 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

Question 37

What causes renal failure in plasma cell disorders?

Answer 37

Precipitated L chains induce interstitial nephritis and hypercalciuria may cause obstructive nephropathy.

Question 38

What is a paraprotein?

Answer 38

Immunoglobulin produced from a single clone of plasma cells, also referred to as M-protein.

Question 39

What is paraproteinaemia?

Answer 39

Presence of a paraprotein in plasma in excessive amounts.

Question 40

What are the categories of paraproteinaemias based on monoclonal protein?

Answer 40

* Light chains only (Bence Jones protein) * Heavy chains only (heavy chain disease) * Whole immunoglobulins (M-protein)

Question 41

What is a Mott cell?

Answer 41

A plasma cell variant with a classic 'bunch of grapes' appearance due to Ig inclusions (Russell bodies).

Question 42

What is the primary cytokine involved in the pathophysiology of multiple myeloma?

Answer 42

Interleukin 6 (IL-6).

Question 43

What are the clinical features of bone pain in multiple myeloma?

Answer 43

Direct effect of osteoclast activating factors and cytokines leading to bone demineralization.

Question 44

What are common symptoms of renal failure in multiple myeloma?

Answer 44

* Polyuria * Polydipsia * Anorexia * Vomiting * Constipation

Question 45

What are the features of anemia in multiple myeloma?

Answer 45

* Lethargy * Weakness * Pallor * Tachycardia

Question 46

What chromosomal abnormality is commonly associated with multiple myeloma?

Answer 46

Abnormalities involving chromosome 14, such as t(11;14), t(4;14), and t(14;16).

Question 47

What is the epidemiology of multiple myeloma in terms of race?

Answer 47

The incidence in blacks is almost twice that in Caucasians.

Question 48

What is the median age at diagnosis for multiple myeloma?

Answer 48

66 years.

Question 49

True or False: Multiple myeloma has a slight male predominance.

Answer 49

True.

Question 50

Fill in the blank: The abnormal plasma cell variant known as _______ has cytoplasm resembling a red flame.

Answer 50

Flame Cell

Question 51

What is TEMPI syndrome?

Answer 51

A syndrome characterized by telangiectasia, elevated erythropoietin, erythrocytosis, monoclonal gammopathy, perinephric fluid collection, and intrapulmonary shunting.