Pathoma - Plasma Cell Disorders, Langerhans Cell Histiocytosis - Fundamentals Of Pathology

Front 1

Plasma cell disorders are often called __________.

Back 1

Plasma cell dyscrasias

Front 2

What is multiple myeloma?

Back 2

Malignant proliferation of plasma cells in marrow.

Front 3

What is the most common primary malignancy of bone?

Back 3

Multiple myeloma

Front 4

In multiple myeloma, what serum molecule is often seen in high concentrations?

Back 4

IL-6

Front 5

IL-6 is an important growth factor for ______________.

Back 5

plasma cells

Front 6

Multiple myeloma plasma cells can activate what other cell type? What is the result?

Back 6

Osteoclasts through their RANK receptor (Osteoclast Activating Factor). They eat away at bone resulting in 'punched-out' lesions seen on X-ray. Increased risk of fracture. Lesions results in bone pain and hypercalcemia.

Front 7

Where do we typically see the 'punched-out' lytic bone lesions associated with multiple myeloma?

Back 7

(1) Vertebrae
(2) Skull

Front 8

The plasma cells of multiple myeloma can produce what?

Back 8

(1) Osteoclast activating factor
(2) Immunoglobulins.

Front 9

A patient has increased serum protein and you can't determine the cause. What could you do?

Back 9

Serum Protein Electrophoresis (SPEP)

Front 10

How is the gamma band on SPEP in normal circumstances? How does it look in multiple myeloma?

Back 10

Wide and not as high as albumin peak.

A sharp tall spike in the gamma region.

Front 11

What does M-spike in multiple myeloma mean?

Back 11

Monoclonal

Front 12

The M-spike on SPEP in multiple myeloma is most commonly due to what Ig?

Back 12

Monoclonal IgG or IgA

Front 13

What is an important complication of multiple myeloma?

Back 13

Monoclonal plasma cells --> Lacks antigenic diversity --> Infection is the MC cause of death

Front 14

What is a complication of multiple myeloma involving having too much protein in the blood?

Back 14

Rouleax formation on smear. Increased serum protein decreases charge between RBCs.

Front 15

Aside from infection and rouleax formation, what is another possible complication of multiple myeloma?

Back 15

Primary AL amyloidosis. Free light chains circulates in serum and deposit in tissue.

Front 16

The proteins in primary amyloidosis associated with multiple myeloma can deposit in tissues. What else can the free chains do?

Back 16

Excreted in urine as Bence-Jones protein (proteinuria). Deposition in kidney tubules leads to risk of renal failure (myeloma kidney).

Front 17

You see a patient in your clinic with a high serum protein. SPEP shows M spike. You suspect MM. You don't find any lytic lesions, hypercalcemia, amyloid or proteinuria. What is going on?

Back 17

MGUS; Monoclonal Gammopathy of Undetermined Significance

Front 18

MGUS is common in the ___________.

Back 18

elderly

Front 19

MGUS is seen in ___% of ____-year-old individuals.

Back 19

5%; 70yo

Front 20

__% of patients with MGUS develop multiple myeloma each year.

Back 20

1%

Front 21

MGUS could almost be described as being a __________.

Back 21

dysplasia

Front 22

What is Waldenströms macroglobulinemia?

Back 22

B-cell LYMPHOMA with monoclonal IgM production (macroglobulin = IgM, because it is big)

Front 23

How do patients with Waldenströms macroglobulinemia present as?

Back 23

(1) Generalized LAD (it is a lymphoma); lytic bone lesions would be absent
(2) Increased serum protein with M spike (comprised of IgM)
(3) Hyperviscosity due to high IgM. Visual and neurologic deficits show (e.g., retinal hemorrhage or stroke).
(4) Bleeding (defective PLT aggregation in hyperviscosity)

Front 24

When patients have acute complications in Waldenstroms macroglobulinemia we can treat them with?

Back 24

Plasmapheresis. Removes IgM from serum.

Front 25

Langerhans cells are _______?

Back 25

Specialized dendritic cells found predominantly in the skin.

Front 26

Langerhans cells are derived from?

Back 26

Bone marrow monocytes

Front 27

Langerhans cell present antigens to?

Back 27

Naive T-cells

Front 28

A tumor of langerhans cells is called?

Back 28

Langerhans cell histiocytosis

Front 29

What is characteristically seen in Langerhans cell histiocytosis?

Back 29

Birbeck graunles on EM (tennis rackets).

Front 30

Cells in langerhans cell histiocytosis have what markers?

Back 30

CD1a+, S100+ by immunohistochemistry.

Front 31

How many subtypes of langerhans cell histiocytosis do we have?

Back 31

3

Front 32

How could you know that a langerhans cell histiocytosis disease is malignant?

Back 32

If the disease is named after someone, the proliferation is usually malignant.

Front 33

Malignant proliferations in langerhans cell histiocytosis usually involve what?

Back 33

The skin. Shows up as a rash.

Front 34

Memory help: If the langerhans cell histiocytosis has two names in it, it is usually seen in?

Back 34

It will usually be seen in children less than 2 (infants)
If there is three peoples names it is usually seen in children greater than the age of 3.

Front 35

Letterer-Siwe classic presentation?

Back 35

Classically presents as skin rash and cystic skeletal defects in an infant (infant < 2 yrs old).

Front 36

What organs are involved in Letterer-Siwe disease? What is the prognosis?

Back 36

Multiple organs may be involved.

Letterer-Siwe is rapidly fatal.

Front 37

Eosinophilic granuloma is a ___________ (benign/malignant) proliferation of _____________ in _________.

Back 37

benign (no name in disease name); Langerhans cells; bone

Front 38

Classic presentation of eosinophilic granuloma?

Back 38

Pathologic fractures in adolescent; skin is not involved (benign, so it does not involve skin)

Front 39

What does a biopsy of a eosinophilic granuloma show?

Back 39

Biopsy shows Langerhans cells with mixed inflammatory cells, including eosinophils.

Front 40

If you got a patient with pathologic fractures that's an adolescent (tenåring, ungdom), you would start to think about what?

Back 40

(1) Osteosarcoma
(2) Eosinophilic granuloma

Front 41

Classic presentation of Hand-Schüller-Christian disease?

Back 41

Classic presentation is scalp rash (malignant, not benign, so it involves skin), lytic skull defects, diabetes insipidus and exopthalmos. Classically seen in people greater than the age of 3.