WBC Disorders Flashcards
Main causes neutropenia
Drug reactions (chemo) Severe infections
Treatment for neutropenia
Treat infection
If chemo induced: GM-CSF or G-CSF
Causes lymphopenia
Immunodeficiency, e.g. DiGeorge
High cortisol states (causes apoptosis lymphos)
AI destruction, e.g. SLE
Whole body radiation
Which cell in body is most sensitive to radiation?
Lymphocytes
Causes neutrophilia
Bacterial infection
Tissue necrosis
High cortisol states: disrupts marginating pool
Left shift
Inflammation causes increased immature neutrophils, marked by fewer Fc receptors (CD16)
Causes monocytosis
Chronic inflamm states
Malignancy
Causes eosinophilia
Neoplasia (HODGKINS--via IL-5 production) Allergy, Asthma Addison's Collagen vascular diseases Parasitic infections
Cause basophilia
CML
Causes lymphocytic leukocytosis
Viral infections
Bordetella pertussis
Infectious mononucleosis
EBV>CMV
Reactive CD8+ cells
Generalized LAD, splenomegaly (PALS), high lympho count with atypical lymphos
Monospot test detecte heterophile IgM Abs; definitive test with EBV viral capsid antigen
Risk splenic rupture–no contact sports 1 yr
Acute leukemia
Blasts >20%
Crowd out normal hematopoiesis so present with signs/sxs cytopenias: anemia, thrombocytopenia, neutropenia
Marker for ALL
Tdt+
Marker for AML
MPO
Which leukemia associated with Down’s after age 5
ALL
Which leukemia associated with Down’s before age 5
Acute megakaryoblastic anemia
Markers B-ALL
CD1-, CD19, CD20
Types B-ALL
t(12;21)-good prog. Kids
t(9;22)-poor prog. Adults
Markers T-ALL
CD2-CD8; do not express CD10
T-ALL presentation
Thymic mass in Teenager
Since causes mass in lymphoid organ, called Acute Lymphoblastic LYMPHOMA
AML types
APML- t(15;17)
Acute monocytic-gums
Acute megakaryoblastic-Downs
Myelodysplastic syndromes
Cytopenias, hypocellular bone marrow
Chronic leukemia
Prolif MATURE lymphocytes
High white count
Insidious
Older adults
CLL
Naive B cell prolif
CD5, CD20
SMudge cells
If goes to LNs=small lymphocytic LYMPHOMA
Can cause hypogammaglobulinemia (thus infection), AIHA, or can transform to diffuse large B cell lymphoma
Hairy cell leukemia
Mature B cell prolif Stains TRAP Hairy cytoplasmic processes Splenomegaly (red pulp), dry tap, NO LAD (trapped in BM) Excellent response to 2-CDA
2-CDA
Adenosine deaminase inhibitor. Adenosine accumulates, which is toxic to B cells
ATLL
Prolif CD4+
Assoc with HTLV
Rash, LAD, HSM, lytic bone lesions with hypercalcemia
Mycosis Fungoides
Prolif CD4+
Rash, plaques, nodules-usu multiple
Neoplastic T cells in epidermis=Pautrier’s microabscesses
If spreads to blood=Sezary’s syndrome
Sezary’s syndrome: classical cell appearance
Cerebriform nuclei
Myeloproliferative Disorders
Accumulation MATURE myeloid cells
Neoplastic proliferation of ALL myeloid cells but named based on predominant cell
Late adulthood
Complications myeloproliferative disorders
Hyperuricemia/gout
Progression to marrow fibrosis
Transformation to acute leukemia
CML
Prolif myeloid cells, esp granulocytes (BASOPHILS)
t(9;22)-BCR-ABL with increased tyrosine kinase activity
Tx: imantinib
CAn transform to AML (2/3) or ALL (1/3)
How do you distinguish CML from leukemoid reaction
CML:
- LAP negative
- t(9;22)
- increased basophils
Polycythemia Vera
Prolif myeloid cells, esp RBCs
JAK2 kinase mutation
Sxs hyperviscosity: blurry vision, HA, Budd-Chiari, flushed face, itching after bathing (mast cells)
Tx with phlebotomy, hydroxyurea
PV versus reactive polycythemia
PV: normal SaO2, decreased EPO
Reactive-lung disease: decreased SaO2, increased EPO
Reactive-ectopic: normal SaO2, increased EPO
Tumors producing ectopic EPO
RCC
HCC
Cerebellar hemangioma
Essential thrombocytopenia
Prolif myeloid cells, esp platelets
JAK2 kinase mutation
Sxs: increased risk bleeding or thrombosis
Does not typically progress to marrow fibrosis or acute leuk
Myelofibrosis
Prolif myeloid cells, esp MKs
JAK2 kinase mutation
MKs produce excess PDGF which causes marrow fibrosis
Splenomegaly once BM fibrosed, leukoerythroblastic smear, increased risk infxn, thrombosis, bleeding
Teardrop cells
T(9;22)
CML on exams
Note that can also be subtype of B-ALL. Adults, poor prognosis
T(15;17)
APML
retinoic acid receptor
T(8;14)
Burkitts
C-myc
T(11;14)
Mantle cell
Cyclin D1
T(14;18)
Follicular lymphoma
Bcl-2
Centroblasts, centrocytes