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Lymphoproliferative neoplasms (Plasma cell myeloma (Px - CRAB (Calcium -…
Lymphoproliferative neoplasms
Plasma cell myeloma
Clonal proliferation of plasma cells in bone marrow, these secrete monoclonal immunoglobulins
Risk factor - more common in men, African Americans, in adults over 50 (70yo)
Px - CRAB
Calcium - hypercalcaemia
Bone pain
Renal insufficiency
Anaemia
Amyloidosis
Hyperviscosity, amylodiosis
Classification
Monoclonal gammopathy of undetermined significance - MGUS
Plasma cell myeloma (multiple myeloma)
Asymptomatic
Symptomatic
Plasmacytoma
Bone - localised bone tumor of monoclonal plasma cells
Extra-osseous - localised plasma cell neoplasm in tissue other than bone
May progress to plasma cell myeloma
Dx
M protein in serum or urine
BM clonal plasma cells, plasma cell infiltration in trephine biopsy - BM plasmacytosis
ROTI - related organ or tissue impairment
Immunophenotyping - CD19+, 20-, 38+, 138+, 56+, 45-, light chain restriction+
Prognostic
B2-microglobulin and albumin
Cytogenetics and FISH
MRI for plasmacytoma of bone
Unfavourable genetics - t(4;14), t(14;16), 17p13 deletions, chr1 abnormalities, 12p deletion
Favourable genetics - hyperdiploidy of 5, 7, 9, 11, 15, 19, 21
Leukemia
Risk factor - above 40, male, previous infection
Px
BM failure - cytopenia
Hepatosplenomegaly, extra-nodal disease, lymphadenopathy
Hairy cell leukemia (HCL)
RF - 50yo M
Px
Massive splenomegaly
Trephine biopsy - marrow fibrosis
Dx
Pancytopenia
Immunophenotype CD11c+, 103+, 25+, 123+
Cytochemistry - TRAP strongly +ve
Genetic mutation BRAF V600E
Chronic lymphocytic leukemia (CLL)
RF - 65yo M
Px
Lymphocytosis, anaemic symptoms, BM symptoms
Immunocompromised, recurrent infection
Constitutional symptoms
Lymphadenopathy, hepatosplenomegaly
Genetics and px
Favourable prognosis - del 13q14.3
Intermediate prognosis - trisomy 12
Poor prognosis - del 17p13 and 11q22 (TP53, ATM)
Richter syndrome - transformation to large cell lymphoma
Immunophenotype - CD19+, 5+, 23+, FMC7, light chain restriction
B lymphocytes interfere with tyrosine kinases and as a result cells don’t die fast enough
T cell prolymphocytic leukemia (T-PLL)
Proliferation of mature T-cells of post-thymic T cell phenotype
RF - 60yo
Dx
Immunophenotype - CD4+, CD8-
Genetics - Inv(14)(q11p32)
Px
Lymphocytosis
Lymphadenopathy
Serous effusions
Adult T-cell leukemia/lymphoma (ATLL)
Mature T cell lymphoma associated with HTLV type 1
RF - 20s to 80s, only adults
WIdespread LN involvement as well as peripheral blood, most with skin involvement
Hypercalcaemia with or without lytic bone lesions
Immunophenotype - CD2+, 3+, 5+, 7-, 25++, 4+, 8-