Plasma cell neoplasms

Question 1

Types of plasma cell neoplasms

Answer 1

Multiple myeloma
Waldenstrom macroglobulinemia
Monoclonal gammopathy of undetermined significance (MGUS)

Question 2

Common cell synthesis feature of plasma cell neoplasms

Answer 2

Monoclonal Ig or Ig fragment

Excess monoclonal light chains as free light chains in plasma

Question 3

Bence-Jones proteins and their test

Answer 3

Free light chains secreted in urine
Tested by urine immunofixation electrophoresis

Question 4

CRAB symptoms of multiple myeloma

Answer 4

Hypercalcemia
Renal failure
Anemia
Lytic bone lesions

Question 5

Plasma cell neoplasm with a higher incidence in older men and those of African descent

Answer 5

Multiple myeloma

Question 6

Translocations/mutation associated with multiple myeloma

Answer 6

t(11;14) - cyclin D1;IgH
t(6;14) - cyclin D3;IgH

MYC mutations

Question 7

Clinical manifestations of multiple myeloma

Answer 7

Pathological fracture
Chronic bone pain
Renal insufficiency/failures
Anemia
Hypercalcemia symptoms
Recurrent bacterial infections
AL type amyloidosis

Question 8

Hypercalcemia symptoms

Answer 8

Weakness
Confusion
Lethargy
Constipation
Polyuria
Renal stones

Question 9

Cause of renal failure in multiple myeloma

Answer 9

Deposition of immunoglobulins within glomeruli and tubular damage

Question 10

Affect of myeloma derived MIP1-alpha

Answer 10

Upregulates RANKL to activate osteoclasts
Increased bone resorption leading to hypercalcemia and fracture

Question 11

AL type amyloidosis

Answer 11

Abnormal processing of free light chains

Question 12

Bone marrow plasma cell morphology in multiple myeloma

Answer 12

Round eccentrically placed nuclei with clumped chromatin

Blue cytoplasm

Perinuclear clearing - golgi

Question 13

Plasma cell leukemia in multiple myeloma

Answer 13

Abnormal bone marrow plasma cells spill into peripheral circulation

Question 14

Immunophenotype of abnormal bone marrow plasma cells in multiple myeloma

Answer 14

CD138/syndecan-1 positive
CD56+

Question 15

Anemia
Normal or decreased WBC and platelets
Elevated ESR
Hypercalcemia
Elevated creatinine and BUN
Urinary excretion of BJP
Monoclonal M protein >3 in serum electrophoresis
Plasma cell infiltration of bone marrow
Lytic lesions

Answer 15

Multiple myeloma

Question 16

Most common plasma cell dyscrasia

Answer 16

Monoclonal gammopathy of undetermined significance (MGUS)

Question 17

Asymptomatic plasma cell neoplasm

Answer 17

MGUS

Question 18

MGUS can progress to this

Answer 18

Multiple myeloma

Question 19

Diagnostic criteria of MGUS

Answer 19

Serum monoclonal protein <3

Clonal plasma cells <10%

Absence of end organ damage

Question 20

B cell neoplasm of older adults

Answer 20

Lymphoplasmacytic lymphoma/Waldenstrom macroglobulinemia

Question 21

Hyperviscosity syndrome mechanism and associated disease

Answer 21

Plasma cell component secretes monoclonal IgM

Waldenstrom macroglobulinemia

Question 22

MYD88 gene mutation

Answer 22

Waldenstrom macroglobulinemia/lymphoplasmacytic lymphoma

Question 23

Clinical features of Waldenstrom macroglobulinemia

Answer 23

Lymphadenopathy
Hepatomegaly
Splenomegaly
Anemia

Question 24

Symptoms of hyperviscosity syndrome

Answer 24

Visual impairment
Neurologic problems (HA, dizziness, stupor)
Bleeding
Cryoglobulinemia

Question 25

Cryoglobulinemia

Answer 25

Precipitation of Ig at low temps Raynaud phenomenon Cold urticaria

Question 26

Normal Langerhans cells

Answer 26

Tissue resident macrophages Form network across epidermis Ability to migrate to draining lymph nodes

Question 27

Typical age of LCH/Histiocytosis X

Answer 27

1-3 yo

Question 28

Types of LCH

Answer 28

Eosinophilic granuloma Hand-Schuller-Christian disease Letterer-Siwe disease Pulmonary LCH

Question 29

General characteristics of eosinophilic granuloma LCH

Answer 29

Unifocal Unisystem, commonly bone Most common in older children and adults

Question 30

General characteristics of Hand-Schuller-Christian disease (LCH)

Answer 30

Multifocal Unisystem

Question 31

Clinical triad in Hand-Schuller-Christian disease

Answer 31

Calvarial bone defects Diabetes insipidus Exophthalmos

Question 32

General characteristics of Letterer-Siwe disease

Answer 32

Multifocal Multisystem Typical age of <2 yo

Question 32

Signs of Letterer-Siwe disease

Answer 32

Erythematous, scaly lesions Hepatosplenomegaly Lymphadenopathy Lytic lesions Recurrent infections

Question 33

Cause of pulmonary LCH

Answer 33

Smoking

Question 34

Prognosis of LCH

Answer 34

Unifocal associated with good prognosis Multifocal and multisystem has poorer prognosis

Question 35

Lesion biopsy findings in LCH

Answer 35

Langerhans cells Eosinophils Lymphocytes Plasma cells Neutrophils

Question 36

Electron micrograph findings in LCH

Answer 36

Birbeck granules

Question 37

Description of Birbeck granules

Answer 37

Rodlike structures with periodicity and dilated terminal end (tennis racket)

Question 38

Most common mutation associated with LCH

Answer 38

Activation mutations involving BRAF

Question 39

Immunophenotype of Langerhans cells

Answer 39

CD1a+ Langerin/CD207+ S-100+