Hematology Lymphoid Flashcards
Normal WBC count
5-10k
Neutropenia can be due to (2)
Drug toxicity
Severe infection
Neutropenia tx
GMCSF
GCSF
Lymphopenia causes (4)
Immunodef (DiGeorge Syndrome failure of thymus form) High cortisol (apoptosis in lympho) Autoimmune destruction (SLE) Whole body radiation (lymphocyte)
Most sensitive cell to radiation
Lymphocyte
Neutrophilic leukocytosis causes (3)
Bacterial i
Tissue necrosis
High cortisol
Inc in immature precursor neutrophils with dec FC receptors that recognize IgG
Left shift
Marker for dec FC receptor
CD16
Disrupts adhesion of neutrophils in marginated pool
cortisol
Monocytosis causes (2)
Chronic inflammation
Maligancy
Eosinophilia causes (3)
Allergic reactions
Parasitic infection
Hodgkin lymphoma
Eosinophilia occurs in hodgkins due to
inc IL5
Basophilia occurs in
CML
Lymphocytic leukocytosis (2)
Viral
Bordetella pertussis
B pertussis produces prevents entry of lymphocytes to LN
Lymphocytosis promoting factor
EBV infection resulting in lymphocytic leukocytosis comprised of reactive CD8 cells
Infectious mononucleosis
EBV transmitted through
saliva
EBV primarily affects
Oropharynx
Liver
B cells
Generalized LAD
Splenomegaly periarterual lymph sheath
High white count of atypical lymphocytes by CD8 cell response
Infectious mononucleosis
EBV infection results in hyperplasia of
paracortex LAD
Which area of white pulp in spleen is affected by EBV
periarterial lymphatic sheath blue in histology
Atypical lymphocytes (2)
Have larger nucleus
Abundant blue cytoplasm
Looks like monocyte
Screening for IM
Monospot test
Detects IgM heterophile (affinity to bind RBC fr other animal) antibodies
Turns positive in 1 week after infection
Negative suggets CMV or early
Definitive diagnostic for IM
EBV viral capsid antigen
IM complications (3)
Inc risk for splenic rupture
Rash if PCN
Dormancy of virus in B cell
EBV inc risk for
Lymphoma in AIDS
Neoplastic proliferation of mature lymphocytes
Chronic leukemia
High WBC count
Insidious onset in older adults
Chronic leukemia
Neoplastic proliferation of B Cells
Chronic lymphocytic leukemia
B cells express (2) in CLL
CD5 and CD20
Inc lymphocytes and SMUDGE cells on blood smear
CLL
Splattered cell in CLL
Smudge cell
Generalized LAD
with small lymphocytic lymphoma (mass)
CLL
CLL Complications (3)
Hypogammaglobulinemia (loss of antibody production)
Autoimmune hemolytic
Transformation to diffuse large B cell lymphoma
MCC of death in CLL
Infection
Neoplastic proliferation of mature B cells
Hairy cytoplasmic processes
+ TRAP
Hairy Cell Leukemia
HCL staining
Tartrate resistant acid phosphatase
Splenomegaly red pulp TRAPED in
Dry tap with bm aspiration
LAD ABSENT
Hairy Cell Leukemia
HCL responds excellently to
2 CDA
Adenosine deaminase (purine degradation pathway) inhibitor Adenosine accumulates to toxic levels in neoplastic B cells
2 CDA
Neoplastic proliferation of mature CD4 T cell
Acute T Cell Leukemia Lymphoma
ATLL associated with
HTLV1 in Japan and carribean (Human T cell leukemia virus 1)
Rash
Generalized LAD with HSM
Lytic bone lesions with hypercalcemia
ATLL
Neoplastic proliferation of mature CD4 T Cell
Mycosis fungoides
Aggregation of T crll in epidermis is called
Pautrier microabscess
CD4+ T cells like to go to
forming
skin
rash, plaque, nodule
Neoplastic CD4 T cells that spread to blood
Characteristic lymphocyte with cerebriform nuclei
Sezary syndrome
Pathognomonic of Sezary syndrome
Lymphocyte with cerebriform nuclei
Accumulation of precursors of myeloid lineage
Acute myelogenous leukemia
Accumulation of immature lymphoid lineage
ALL
Accumulation of mature lymphoid cells
CL
Accumulation of mature myeloid cells
Myeloproliferative disorders
Overproduction of rbcs
Polycythemia vera
Disease of late adulthood
High WBC count
Hypercellular marrow
Cells of all lineages inc
Myeloproliferative disorder
Myeloproliferative disorder complications (3)
1 hyperuricemia gout
2 marrow fibrosis
3 acute leukemia
Neoplastic proliferation of mature myeloid cells esp basophils
Chronic Myeloid Leukemia
CML translocation
t 9;22 BCRL ABL fusion with inc tyrosine kinase activity leading to overgrowth
Tx for CML
Imatinib
blocks tk activity
Phases of CML (3)
Chronic phase enlarged spleen suggesting acceleration worsening
Acceleration phase
Transformation phase - Acute Leukemia either AML or ALL bec mutation is in HSC
PCV tx (2)
Phlebotomy
Hydroxyurea
Tumor that produces EPO ectopically
Renal cell carcinoma
Proliferation of mature myeloid esp platelets
Associated with JAK2 kinase mutation
Essential thrombocythemia
IDA can also have inc
Platelet
Differentiate from essential thrombocythemia
Inc risk of bleeding/thrombosis
Rarely progresses to marrow fibrosis or acute leukemia
No significant risk for hyperuricemia or gout
Essential thrombocythemia
Neoplastic proliferation of mature myeloid cells megakaryocytes
Jak2 mutation
Myelofibrosis
Marrow fibrosis occurs in myelofibrosis because of
PDGF
Splenomegaly
Leukoerythroblastic smear (reticulin gates prohibit immature cells from exiting to blood, does not occur in spleen)
Inc infection, thrombosis bleeding
Myelofibrosis
Pathognomonic of myelofibrosis
tear drop cell
Painful LAD
acute
painless LAD
chronic, metastatic, lymphoma
Rheumatoid arthritis and HIV
follicle enlargement
Viral infection
Paracortex englargement
LN draining tissue with cancer
Sinus histoocytes
Neoplastic prolif of lymphoid cells that form a mass
Arise in LN and extra nodal tissue
Lymhpoma
60% of lymphomas
NHL
40% of Lymphoma
HL
Small neoplastic B cells (4)
Follicular
Mantle
Marginal
Small lymphocytic lymphoma
Neoplastic proliferation of small B cells CD20 making follicle like nodules
Presents in late adulthood painless LAD
Follicular lymphoma
Follicular lymphoma is driven by
t 14;18
BCL2 on ch18 translocates to Ig heavy chain locus on ch14
Overexpression of bcl2 inhibiting apoptosis
Tx for Follicular lymphoma
Rituximab antiCD20
Chemotherapy
Follicular lymphoma complication (2)
Progress to diffuse large B cell lymphoma
Enlarging lymph node
Follicular lymphoma is differentiated from hyperplasia by (4)
1 disruption of LN architecture
2 lack of tingible body macrophage in germinal center
3 monoclonality (ratio of kappa to lambda light chain disruptive 20:1
Neoplastic proliferation of small b cells that expand mantle zone
Late adulthood
painless LAD
expands region adjacent to follicle
Mantle Cell Lymphoma
Mantle Cell Lymphoma is driven by
t 11;14
Cyclin D1 (helps cell go from G1 to S) phosphorylation on ch 11 translocates to Ig heavy chain ch 14
Neoplastic small B cells that expand marginal zone
Marginal zone lymphoma
Mantle B cell lymphoma is associated with (3)
Chronic inflamm states
Hashimoto, Sjogren’s, H pylori
Post germinal B cells form Marginal zone
Marginal zone lymphoma in mucosal site
MALToma
Neoplastic intermediate-sized B cells CD20
Associated with EBV
Burkitt lymphoma
Burkitt lymphoma (2)
African form jaw
Sporadic form abdomen!
Burkitt lymphoma is driven by
t 8;14 translocation of c-myc (nuclear regulator activating multiple genes resulting to a lot of growth) to Ig heavy chain locus on ch14
Burkitt has high mitotic rate pathognomonic of
Starry sky on histology
Neoplastic large B cells that grow diffusely in sheet
Diffuse large b cell lymphoma
Most common form of non hodgkin lymphoma
Aggressive
DLBCL
DLBCL arise (2)
sporadically
transformation of follicular lymphoma
Pathognomonic of Hodgkin
Reed sternberg cell
Neoplastic proliferation of reed sternberg cell which are large b cell with multilobed nuclei and prominent nucleoli
CD15 CD30
Hodgkin lymphoma
Reed sternberg cell
Owl eye nucleus
RS cells secrete
Cytokine
Hence with B symptoms
Attract lympho mac plasma eo (mass formation)
Fibrosis
Basis of subtype of Hodgkin
type of reactive cellularity
70% of cases of hodgkin
Nodular sclerosis
Hodgkin variants (4)
Nodular sclerosis
Lymphocyte rich
Mixed
Lymphocyte depleted
Enlarging cervical neck or mediastinal LN in young adult female
Nodular sclerosis
Nodular sclerosis Hodgkin biopsy
Broad pink bands of fibrosis dividing lymph nides with RS cells in lake-like spaces lacunar cell
Hodgkin with best prognosis
Lymphocyte rich
Hodgkin with worst prognosis in elderly and HIV+
Lymphocyte-depleted
Abundant in the mixed cellularity type
eosinophil by il-5
Malignant proliferation of plasma cell in bm
Multiple myeloma
Most common primary malignancy of bone driven by
Multiple myeloma
IL 6
Pathognomonic of MM
Punched out lesions on xray
Bec of activation of RANK on osteoclast
Bone pain with hypercalcemia
Elevated serum protein Immuneglobin
Sharp spike M spike in gamma region of serum electrophoresis
Indicating monoclonal immunoglobulin (IgG or IgA)
Multiple myeloma
Multiple myeloma inc risk of infection bec
(1) lack antigenic diversity
Most common cause of death
2 dec charge in RBC - roleaux formation
Circulation and overproduction of free light chain in serum depositing in tissue
Primary AL amyloidosis
Free light chain excreted in urine
Bence Jones protein
Deposition in kidney leads to risk of myeloma kidney
Inc serum protein M spike on SPEP No lytic lesion No hypercalcemia No amyloid No bence jones protein
Monoclonal Gammopathy of Undetermined Significance
MGUS is common in
1% develops
elderly
Multiple myeloma
B cell lymphoma with monoclonal IgM production
Waldenstrom Macroglobulinemia
LAD no lytic bones Inc serum protein M spike IgM Visual and neuro deficits (inc viscosity) Bleeding
Waldenstrom Macroglobulinemia
WM complications tx
Plasmapharesis
Specialized dendritic cells in skin
From bone marrow monocytes
Present antigen to naive T cell
Langerhan cells
Neoplastic proliferation of LH crlls
Langerhan cell histiocytosis
LH pathognomonic for (2)
Birbeck tennis racket granules on em
CD1a+ S100+ IHC
Malignant proliferation of LH cell
Skin rash and cystic skeletal defect in an infant
Multiple organ
Rapidly fatal
Letterer Siwe disease
Benign proliferation of LH cell in bone
Pathologic fracture no skin involvement
Langerhan cells with mixed inflamm and eosinophilia
Eosinophilia granuloma
Malignant proliferation of LH cell
Scalp, rash, lytic skull, diabetes insipidus, exopthalmos
child
Hand-Schuller-Christian