6.8,9 Plasma Cell Dyscrasias, Langerhans Histiocytosis Flashcards
multiple myeloma
-clinical presentation (6)
- Osteoclast inhibiting factor–bone lesions with hypercalcemia
- elevated monoclonal Ab–increased risk of infection
- elevated monoclonal Ab–Rouleaux formation of RBCs b/c increased serum protein decreases charge btwn RBCs
- free light chains–primary AL amyloidosis
- free light chains–Bence Jones protein in urine
- free light chains–renal failure (myeloma kidney)
What is SPEP and how is it used in multiple myeloma?
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
Langerhans cell histiocytosis
-subtypes (3)
- 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
Letterer-Siwe disease
- classic presentation
- population
- a Langerhans cell histiocytosis
- infants
- malignant proliferation of Langerhans cells. skin rash, cystic skeletal defects. multiple organs can be involved; rapidly fatal
Pt with bone lesions, renal failure, infection.
-Suspect what
-Multiple myeloma
MGUS
Monoclonal gammopathy of undetermined significance:
- what population?
- complications
- common in elderly (5% of 70 yo’s)
- 1% of them develop multiple myeloma each year
List plasma cell dyscrasias (3)
- multiple myeloma
- MGUS (monoclonal gammopathy of undetermined significance)
- waldenstrom macroglobulinemia
multiple myeloma
-mech of its effect on bone
- Plasma cells produce osteoclast activating factor, which binds to RANK receptor on osteoclasts, activating them.
- causes ‘punched-out’ bone lesions with hypercalcemia
Adolescent pt presents with bone fx from playing basketball.
- 1st thought, suspect what neoplastic disorder?
- what else to keep in differential?
- suspect osteosarcoma
- also consider Eosinophilic granuloma
which immunoglobulins are overproduced in multiple myeloma?
in Wadenstrom macroglobulinemia?
- IgG or IgA
- IgM
Child with rash on head, skull defects, increased urination, and exophthalmos.
-Think what?
-Hand-Schuller-Christian disease
(Langerhans cell histiocytosis)
most common cause of death in multiple myeloma
Infection
-increased risk of infection in multiple myeloma b/c monoclonal Abs lack antigenic diversity
Wadenstrom macroglobulinemia
-tx
-plasmapherisis: remove blood to remove IgM, then return blood
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?
MGUS
Monoclonal gammopathy of undetermined significance
Hand-Schuller-Christian disease
- classic presentation (4 symptoms)
- population
- malignant proliferation of Langerhans cells
- children >3
- scalp rash, lytic skull defects, diabetes insipidus, exophthalmos
multiple myeloma
-how can it affect the kidney
-overproduction of light chain means it can deposit in the kidney tubules, causing kidney failure
(myeloma kidney)
Pt with generalized LAD, also with retinal hemorrhage and stroke.
Suspect what?
Suspect Waldenstrom macroglobulinemia
multiple myeloma
-why increased risk of infection
-Plasma cells produce monoclonal Ab, which lack antigenic diversity.
Pt is an infant with skin rash and cystic skeletal defects.
Think what?
Letterer-Siwe disease–Langerhans cell histiocytosis
Pt you suspect has multiple myeloma:
-what symptoms, findings to look for (4)
- bone lesions
- hypercalcemia
- AL amyloid
- Bence-Jones proteinuria
multiple myeloma
-assoc with what cytokine? what does it do?
IL-6
-stimulates plasma cell growth and immunoglobulin producion
Wadenstrom macroglobulinemia
-what is this?
-B cell lymphoma with monoclonal IgM production (as opposed to multiple myeloma with IgG and IgA production)
Wadenstrom macroglobulinemia
-clinical findings (4)
- Generalized LAD, no bone lesions
- increased serum protein with M spike (IgM)
- visual and neurologic defects (eg retinal hemorrhage, stroke): serum hyperviscosity from large IgM
- Bleeding–high viscosity results in defective platelet aggregation.
MGUS
Monoclonal gammopathy of undetermined significance:
-what is this
-When a pt has increased serum protein with M spike, but no other features of multiple myeloma
Langerhans cell histiocytosis
- what are langerhans cells?
- appearance on electron microscopy
- what cell markers?
- 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+
multiple myeloma
what products are elevated in the blood? (3)
- osteoclast activating factor
- monoclonal Abs
- free light chains
what is the most common primary malignancy of bone?
multiple myeloma
Eosinophilic granuloma
- classic presentation
- population
- What you see on biopsy
- Benign proliferation of Langerhans cells in bone
- adolescents, pathological fractures. (eg playing sports)
- Biopsy shows Langerhans cells with mixed inflammatory cells, including numerous eosinophils
multiple myeloma
-proliferation of what
-proliferation of plasma cells in bone marrow
How does overproduction of light chain present as symptoms in multiple myeloma?
- Primary AL amyloidosis–light chain deposits in tissues
- light chain in urine (Bence Jones protein)
- light chain deposits in tubules. (myeloma kidney–renal failure)
multiple myeloma
-appearance on blood smear
- Rouleaux formation of RBCs (RBCs are stacked like poker chips)
- b/c increased serum protein decreases the charge between RBCs
