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6 White Blood Cell disorders > 6.8,9 Plasma Cell Dyscrasias, Langerhans Histiocytosis > Flashcards

6.8,9 Plasma Cell Dyscrasias, Langerhans Histiocytosis Flashcards

1
Q

multiple myeloma

-clinical presentation (6)

A
  1. Osteoclast inhibiting factor–bone lesions with hypercalcemia
  2. elevated monoclonal Ab–increased risk of infection
  3. elevated monoclonal Ab–Rouleaux formation of RBCs b/c increased serum protein decreases charge btwn RBCs
  4. free light chains–primary AL amyloidosis
  5. free light chains–Bence Jones protein in urine
  6. free light chains–renal failure (myeloma kidney)
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2
Q

What is SPEP and how is it used in multiple myeloma?

A

SPEP: serum protein electrophoresis. Used to determine amount of different proteins in the blood.

-in multiple myeloma, you would see an ‘M spike’ for globulin amount, indicating increased immunoglobulin production by plasma cells

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2
Q

Langerhans cell histiocytosis

-subtypes (3)

A
  • use mnemonic: If it has peoples’ names, it is malignant and involves skin. If 2 names, then in children 3.
    1. Letterer-Siwe Disease
  • malignant, has skin rash, children
    2. Eosinophilic granuloma
  • benign, no skin rash, adolescents with pathologic bone fx’s
    3. Hand-Schuller-Christian disease
  • malignant, has skin rash, children >3
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2
Q

Letterer-Siwe disease

  • classic presentation
  • population
A
  • a Langerhans cell histiocytosis
  • infants
  • malignant proliferation of Langerhans cells. skin rash, cystic skeletal defects. multiple organs can be involved; rapidly fatal
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3
Q

Pt with bone lesions, renal failure, infection.

-Suspect what

A

-Multiple myeloma

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4
Q

MGUS

Monoclonal gammopathy of undetermined significance:

  • what population?
  • complications
A
  • common in elderly (5% of 70 yo’s)
  • 1% of them develop multiple myeloma each year
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4
Q

List plasma cell dyscrasias (3)

A
  1. multiple myeloma
  2. MGUS (monoclonal gammopathy of undetermined significance)
  3. waldenstrom macroglobulinemia
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5
Q

multiple myeloma

-mech of its effect on bone

A
  • Plasma cells produce osteoclast activating factor, which binds to RANK receptor on osteoclasts, activating them.
  • causes ‘punched-out’ bone lesions with hypercalcemia
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6
Q

Adolescent pt presents with bone fx from playing basketball.

  • 1st thought, suspect what neoplastic disorder?
  • what else to keep in differential?
A
  • suspect osteosarcoma
  • also consider Eosinophilic granuloma
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7
Q

which immunoglobulins are overproduced in multiple myeloma?

in Wadenstrom macroglobulinemia?

A
  1. IgG or IgA
  2. IgM
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7
Q

Child with rash on head, skull defects, increased urination, and exophthalmos.

-Think what?

A

-Hand-Schuller-Christian disease

(Langerhans cell histiocytosis)

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9
Q

most common cause of death in multiple myeloma

A

Infection

-increased risk of infection in multiple myeloma b/c monoclonal Abs lack antigenic diversity

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10
Q

Wadenstrom macroglobulinemia

-tx

A

-plasmapherisis: remove blood to remove IgM, then return blood

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11
Q

Pt has increased serum protein with M spike on SPEP, but no bone lesions, AL amyloid, hypercalcemia, or Bence Jones proteinuria.

What does this pt have?

A

MGUS

Monoclonal gammopathy of undetermined significance

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11
Q

Hand-Schuller-Christian disease

  • classic presentation (4 symptoms)
  • population
A
  • malignant proliferation of Langerhans cells
  • children >3
  • scalp rash, lytic skull defects, diabetes insipidus, exophthalmos
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12
Q

multiple myeloma

-how can it affect the kidney

A

-overproduction of light chain means it can deposit in the kidney tubules, causing kidney failure

(myeloma kidney)

13
Q

Pt with generalized LAD, also with retinal hemorrhage and stroke.

Suspect what?

A

Suspect Waldenstrom macroglobulinemia

14
Q

multiple myeloma

-why increased risk of infection

A

-Plasma cells produce monoclonal Ab, which lack antigenic diversity.

15
Q

Pt is an infant with skin rash and cystic skeletal defects.

Think what?

A

Letterer-Siwe disease–Langerhans cell histiocytosis

16
Q

Pt you suspect has multiple myeloma:

-what symptoms, findings to look for (4)

A
  1. bone lesions
  2. hypercalcemia
  3. AL amyloid
  4. Bence-Jones proteinuria
18
Q

multiple myeloma

-assoc with what cytokine? what does it do?

A

IL-6

-stimulates plasma cell growth and immunoglobulin producion

19
Q

Wadenstrom macroglobulinemia

-what is this?

A

-B cell lymphoma with monoclonal IgM production (as opposed to multiple myeloma with IgG and IgA production)

20
Q

Wadenstrom macroglobulinemia

-clinical findings (4)

A
  1. Generalized LAD, no bone lesions
  2. increased serum protein with M spike (IgM)
  3. visual and neurologic defects (eg retinal hemorrhage, stroke): serum hyperviscosity from large IgM
  4. Bleeding–high viscosity results in defective platelet aggregation.
22
Q

MGUS

Monoclonal gammopathy of undetermined significance:

-what is this

A

-When a pt has increased serum protein with M spike, but no other features of multiple myeloma

23
Q

Langerhans cell histiocytosis

  • what are langerhans cells?
  • appearance on electron microscopy
  • what cell markers?
A
  • Dendritic cells (derived from bone marrow monocytes), found in skin. are antigen presenting cells to naive T cells.
  • tennis racket granules (Birbeck granules)
  • CD1a+, S100+
24
Q

multiple myeloma

what products are elevated in the blood? (3)

A
  1. osteoclast activating factor
  2. monoclonal Abs
  3. free light chains
26
Q

what is the most common primary malignancy of bone?

A

multiple myeloma

27
Q

Eosinophilic granuloma

  • classic presentation
  • population
  • What you see on biopsy
A
  • Benign proliferation of Langerhans cells in bone
  • adolescents, pathological fractures. (eg playing sports)
  • Biopsy shows Langerhans cells with mixed inflammatory cells, including numerous eosinophils
29
Q

multiple myeloma

-proliferation of what

A

-proliferation of plasma cells in bone marrow

30
Q

How does overproduction of light chain present as symptoms in multiple myeloma?

A
  1. Primary AL amyloidosis–light chain deposits in tissues
  2. light chain in urine (Bence Jones protein)
  3. light chain deposits in tubules. (myeloma kidney–renal failure)
31
Q

multiple myeloma

-appearance on blood smear

A
  • Rouleaux formation of RBCs (RBCs are stacked like poker chips)
  • b/c increased serum protein decreases the charge between RBCs

6 White Blood Cell disorders (6 decks)

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