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HML II > Plasma cell neoplasms > Flashcards

Plasma cell neoplasms Flashcards

1
Q

Types of plasma cell neoplasms

A

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

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2
Q

Common cell synthesis feature of plasma cell neoplasms

A

Monoclonal Ig or Ig fragment

Excess monoclonal light chains as free light chains in plasma

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3
Q

Bence-Jones proteins and their test

A

Free light chains secreted in urine
Tested by urine immunofixation electrophoresis

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4
Q

CRAB symptoms of multiple myeloma

A

Hypercalcemia
Renal failure
Anemia
Lytic bone lesions

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5
Q

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

A

Multiple myeloma

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6
Q

Translocations/mutation associated with multiple myeloma

A

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

MYC mutations

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7
Q

Clinical manifestations of multiple myeloma

A

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

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8
Q

Hypercalcemia symptoms

A

Weakness
Confusion
Lethargy
Constipation
Polyuria
Renal stones

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9
Q

Cause of renal failure in multiple myeloma

A

Deposition of immunoglobulins within glomeruli and tubular damage

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10
Q

Affect of myeloma derived MIP1-alpha

A

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

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11
Q

AL type amyloidosis

A

Abnormal processing of free light chains

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12
Q

Bone marrow plasma cell morphology in multiple myeloma

A

Round eccentrically placed nuclei with clumped chromatin

Blue cytoplasm

Perinuclear clearing - golgi

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13
Q

Plasma cell leukemia in multiple myeloma

A

Abnormal bone marrow plasma cells spill into peripheral circulation

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14
Q

Immunophenotype of abnormal bone marrow plasma cells in multiple myeloma

A

CD138/syndecan-1 positive
CD56+

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15
Q

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

A

Multiple myeloma

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16
Q

Most common plasma cell dyscrasia

A

Monoclonal gammopathy of undetermined significance (MGUS)

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17
Q

Asymptomatic plasma cell neoplasm

A

MGUS

18
Q

MGUS can progress to this

A

Multiple myeloma

19
Q

Diagnostic criteria of MGUS

A

Serum monoclonal protein <3

Clonal plasma cells <10%

Absence of end organ damage

20
Q

B cell neoplasm of older adults

A

Lymphoplasmacytic lymphoma/Waldenstrom macroglobulinemia

21
Q

Hyperviscosity syndrome mechanism and associated disease

A

Plasma cell component secretes monoclonal IgM

Waldenstrom macroglobulinemia

22
Q

MYD88 gene mutation

A

Waldenstrom macroglobulinemia/lymphoplasmacytic lymphoma

23
Q

Clinical features of Waldenstrom macroglobulinemia

A

Lymphadenopathy
Hepatomegaly
Splenomegaly
Anemia

24
Q

Symptoms of hyperviscosity syndrome

A

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

25
Q

Cryoglobulinemia

A

Precipitation of Ig at low temps
Raynaud phenomenon
Cold urticaria

26
Q

Normal Langerhans cells

A

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

27
Q

Typical age of LCH/Histiocytosis X

A

1-3 yo

28
Q

Types of LCH

A

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

29
Q

General characteristics of eosinophilic granuloma LCH

A

Unifocal
Unisystem, commonly bone

Most common in older children and adults

30
Q

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

A

Multifocal
Unisystem

31
Q

Clinical triad in Hand-Schuller-Christian disease

A

Calvarial bone defects
Diabetes insipidus
Exophthalmos

32
Q

General characteristics of Letterer-Siwe disease

A

Multifocal
Multisystem
Typical age of <2 yo

32
Q

Signs of Letterer-Siwe disease

A

Erythematous, scaly lesions
Hepatosplenomegaly
Lymphadenopathy
Lytic lesions
Recurrent infections

33
Q

Cause of pulmonary LCH

A

Smoking

34
Q

Prognosis of LCH

A

Unifocal associated with good prognosis

Multifocal and multisystem has poorer prognosis

35
Q

Lesion biopsy findings in LCH

A

Langerhans cells
Eosinophils
Lymphocytes
Plasma cells
Neutrophils

36
Q

Electron micrograph findings in LCH

A

Birbeck granules

37
Q

Description of Birbeck granules

A

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

38
Q

Most common mutation associated with LCH

A

Activation mutations involving BRAF

39
Q

Immunophenotype of Langerhans cells

A

CD1a+
Langerin/CD207+
S-100+

HML II (15 decks)

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