Lymphoproliferative Disorders Flashcards
Lymphoproliferative disorder definition
group of malignant or benign neoplastic lesions of the lymphoid system
includes leukemias, lymphomas & disorders
LYMPHOID line including plasma cells
chronic, usually increased WBC & accumulation of malignant lymphs
Lymphoproliferative disorders
- CLL
- Prolymphocytic Leukemia
- Hairy cell leukemia
- multiple myeloma
- Waldenstrom’s macroglobulinemia
- hodgkins lymphoma
- non-hodgkin’s lymphoma
Prolymphocytic Leukemia
rare, aggressive disorder
doesn’t respond to treatment
70% are B cell linage
predominant in males
Prolymphocytic Leukemia lab findings
marked lymphocytosis - 55% of lymphs are prolymphocytes (large lymphs w/ nucleoli, CD19+)
anemia
thrombocytopenia
Hairy Cell Leukemia (HCL)
rare, malignancy of B cells
does NOT develop into acute leukemia
Epidemiology of Hairy Cell Leukemia
middle aged patients
more common in males than females (7:1)
mutation on x chromosome may play a role
Hallmark of HCL
pancytopenia Hairy lymphocytes! abundant pale cytoplasm w/ cytoplasmic projections oval nuclei fine chromatin
Symptoms of HCL
usually massive splenomegaly
weakness
fatigue
bruising
Lab findings of HCL in PB
pancytopenia!
normo, normo anemia
presence of hairy cells - positive w/ TRAP cells
CD19+, CD20+, CD22+, CD25+, CD11c +
Lab findings of HCL in BM
DRY TAP bc of hypercellularity
HCL is associated w/ increased amounts of reticulin
Multiple Myeloma
malignancy of plasma cells in the BM male predominance (65 yrs)
Etiology of Multiple Myeloma
genetic mutation that causes cytokine IL-6 stimulation in the BM that results in excess plasma cells
interfere w/ production of other blood cells & immune system
Characterization of MM
excess plasma cells -> excess antibodies -> excess protein (IgG maybe IgA) -> thick blood -> excess protein (light chains) excreted through the urine
Bence-Jones protein
excess antibody light chains in the urine
positive in Multiple Myeloma
Hallmark of MM
increase in plasma cells
ROULEAUX in PB
BONE LESIONS & PAIN
gamma spike on serum electrophoresis
Symptoms of MM
bone pain & fracture - ‘punched out lesions’ of bones on x-rays
IL-6 is an activator of bone dissolvent
kidney issues
PB lab findings of MM
leukocytosis - plasma cells anemia ROULEAUX blue tinge to the slide (excess protein) increased ESR Bence-Jones protein in urine increased serum protein - gamma spike
BM lab findings of MM
hypercellular
increased # of plasma cells
mott cells & flame cells present
MM prognosis
w/o treatment 6 months
w/ treatment average is 3 years
Lymphoma
general term for cancers that start in the lymphatic system
Hodgkins & non-Hodgkins
Causes of Lymphomas
gene mutations
environmental - chemical exposure
viruses - HIV, epstein-barr
idiopathic
Diagnosis of Lymphomas
lymph node biopsy
imaging studies
flow cytometry immunophenotyping
Hodgkin’s Lymphoma
12% of lymphomas , higher in adolescents & young adults
painless swelling of lymph nodes in neck/armpit/groin
recurrent high fever
Reed-Sternberg cells!
Reed-Sternberg cells
characteristic of Hodgkin’s lymphoma
extremely large, abnormal lymphs
bi-nucleus (looks like 2 attached nuclei)
Hodgkin’s prognosis
one of the most curable forms of cancer
Non-Hodgkin’s Lymphomas
B-cell lymphomas or T-Cell lymphomas
B more common than T
lack Reed-Sternberg cells, resemble normal lymphoid cells
Waldenstrom’s Macroglobulinemia
AKA lymphoplasmacytic lymphoma
similar to MM, but w/ IgM
affects >65 yrs.
no bone lesions or pain
Waldenstrom’s Macroglobulinemia Lab findings
leukocytosis - malignant cells are mixed population (lymphs & possibly plasma cells)
anemia
ROULEAUX
elevated IgM on the monoclonal spike of SPE
Small Lymphocytic Lymphoma
tissue equivalent of CLL
biopsy shows diffuse infiltrate of small mature lymphocytes
Follicular Lymphoma
most common type of non-Hodgkin lymphoma
50-60 yrs
biopsy shows ‘nodular pattern’ of lymphoid follicle
CD19+, CD20+, CD5 -, sIg strong
Mantle Cell Lymphoma
male predominance (> 60 yrs) present w/ disseminated disease involving lymph, skeletal, spleen, liver, GI systems multiple polyps involving small intestine
Mantle Cell Lymphoma Immunophenotype
CD19+, CD20+, CD5+, CD23- , FMC-7 +, sIg +
relatively aggressive lymphoma
Burkitt Lymphoma
high incidence in Africa
HIV patients
biopsy shows ‘starry sky’
‘Starry Sky’ appearance
characteristic of Burkett’s Lymphoma
sky - blue nuclei of neoplastic lymphs
stars - scattered pale-staining body macrophages
Lymphoblastic Lymphoma
tissue equivalent of ALL
80% T cells
children affected > adults
males > females
