PCD Flashcards
mature B cell capable of synthesizing antibodies
PLASMA CELL
are proteins with gamma electrophoretic mobilities that are synthesized in response to an antigen
ANTIBODIES (IMMUNOGLOBULINS or g GLOBULINS) –
Activators of classical complement pathway
IgG
Hemaglutinins
Hemolysins
IgG
Most common (80%)
IgG
Precipitating antibody
Virus-neutralizing antibody
IgG
Synthesized by plasma cells found in the respiratory and GI tracts and most excretory organs
IgA
First line of defense for mucosal surfaces
IgA
Second most abundant (10-15%)
IgA
First antibody to appear in response to an antigenic challenge
IgM
Circular pentameric arrangement
IgM
Largest antibody
IgM
5-10% of circulating antibodies
Localized in blood
IgM
“Ragweed” antibody
IgD
Trace concentrations
IgD
MW = 140,000 daltons
IgD
Functions in lymphocytic activation and suppression
IgD
Mediates allergic reactions and parasitic infections
IgE
Attaches to mast cells and basophils
IgE
“Reagenic antibody”
IgE
detects imbalance of Ig of free H or L chains
IMMUNOELECTROPHORESIS and IMMUNOFIXATION ELECTROPHORESIS
Most common disease of plasma cells
MULTIPLE MYELOMA
MULTIPLE MYELOMA
Predisposing factors
Genetics
Chronic antigenic stimulation
Begins in the bone marrow with lytic bone diseases and osteoporosis
MULTIPLE MYELOMA
Igs produced are mostly of the _ class (50%) or _ (20%) or L-chains only __ (15%) or _ (25%)
IgG
IgA
(Bence-Jones Protein)
Amyloid
CLINICAL PRESENTATION
HyperCalcemia
Renal insufficiency
Anemia
Bone lesions
LABORATORY FINDINGS
N/N anemia
ESR elevated due to increased globulins
Rouleaux formation
Few abnormal plasma cells
BM = 10-15% plasmacytes
Urinary protein (strip and SSA) – not sensitive due to their specificity to albumin
(+) casts – hyaline or tubular epithelial casts
Electrophoresis – monoclonal peak
Hyperuricemia
Hypercalcemia
Radiologic exams
MYELOMA CELL CHARACTERISTICS
Large with immature appearing chromatin OR small with clumped chromatin
Pale or dark (depending on cytoplasmic RNA)
Sometime bizarre and multinucleated or lobulated nuclei
Inclusions
Accumulations of Igs in the cisternae
MOTT CELL, MORULA CELL or PLASMA CELL WITH RUSSELL BODIES
intranuclear inclusions
DUTCHER BODIES –
Circulating plasma cell levels > 2 X 109/L (0.14-0.20 NV)
PLASMA CELL LEUKEMIA
PLASMA CELL LEUKEMIA
Younger patients
Less bone pain
Less osteolysis
Greater incidence of lymphadenopathy and hepatosplenomegaly
Pancytopenia with
leukoerythroblastic findings
Elevated ESR
Abnormal plasma cells are smaller with little cytoplasm and pronounced N:C asynchronism
BM infiltration is diffuse (45%)
A lymphoproliferative disorder characterized by large concentrations of monoclonal IgM (>15%)
WALDENSTROM MACROGLOBULINEMIA
The abnormal B lymphocytes arise from the extramedullary nodes and have the ability to differentiate to large plasmacytoid lymphocytes and plasma cells
WALDENSTROM MACROGLOBULINEMIA
Rarely causes osteolytic lesions
WALDENSTROM MACROGLOBULINEMIA
Glomerular lesions caused by deposition of IgM complexes of amyloid
WALDENSTROM MACROGLOBULINEMIA
Affects men >50 years old with hepatosplenomegaly, lymphadenopathy and retinal abnormalities
WALDENSTROM MACROGLOBULINEMIA
WALDENSTROM MACROGLOBULINEMIA
LABORATORY FINDINGS
N/N
Reticulocytes dec. or N
Normal WBC and platelets
BM- plasmacytoid lymphoma
(+) Mast cells – CLUE!
Autoimmune hemolytic anemia
Raynaud’s phenomenon (anti-G)
Cold agglutinin (anti-i)
WALDENSTROM MACROGLOBULINEMIA
platelet function
fibrinogen
thrombin time
plasma viscosity
Monoclonal IgM with cryoglobulin activity
Abnormal platelet function
Thrombocytosis (maybe plasma precipitates)
Low fibrinogen
Prolonged thrombin time
Increased plasma viscosity
– precipitation or gel formation at 4C
Electrophoresis
A rare disease characterized by abnormal synthesis of Fc fragment of a particular H chain particularly a (most common), g and m
HEAVY CHAIN DISEASE
Resembles activated lymphocytes and plasma cell
Lymphoma like symptoms
HEAVY CHAIN DISEASE
HCD
characterized by infiltration of plasmacytoid lymphocytes into the duodenal and jejunal walls producing malabsorption and abdominal distress and respiratory tracts
ALPHA HCD
HCD Found in older men and presents with a malignant lymphoma with fever, erythema, lymphadenopathy and hepatosplenomegaly
GAMMA HCD
HCD Rarest; associated with CLL
MU HCD
A condition in which proetinaceous deposits (amyloid) occur throughout the body
AMYLOIDOSIS
closely associated with monoclonal gammopathies such as plasma cell myeloma
PRIMARY AMYLOIDOSIS
associated with chronic illnesses and amyloid unrelated to Ig
SECONDARY AMYLOIDOSIS
AMY Electron microscopy and Xray diffraction
amyloid is in the Beta pleated sheet formation
AMY Polarizing microscopy with Congo red
apple green birefringence