Abnormal WBCs and Leukemia Flashcards
Diapedesis
Passage of blood cells through intact capillary walls, usually accompanying inflammation
T Cell Markers
Tdt, CD4, CD8
B Cell Markers
Surface Ig, CD34, CD19
Hypersegmentation occurs in
megaloblastic anemia or in response to infection
Hyposegmentation occurs in
Pelger Huet Anomaly, Myeloproliferative Disorder (MPS) or Myelodysplastic Disorder (MDS)
Dohle Bodies
Light blue granular inclusions, which are aggregates of ER (RNA), associated with infection and May-Hegglin anomaly
Auer Rods
Red or orange needle-like inclusions, made of fused lysosomes. Associated with myelocytic leukemia
Russell Bodies
“Flame Cells” (stark cytoplasmic color gradient) and “Mott Cells” (extreme vacuolation)
Barr Bodies
“Drumstick” (tiny nuclear lobe) inclusions made of inactive X chromosomes. Seen in females and Klinefelters syndrome patients
Leukemoid Reaction
Leukemia-like response to chronic infection. Severe left-shift, usually >25 x10^9/L neutrophils. Distinguished with high LAP score, toxic granulation, and Dohle Bodies from Chronic Myelogenous Leukemia (CML).
Marginated Granulocyte Pool (MGP)
Neutrophils that are stuck to the inside of the vascular system and don’t come out w/ peripheral blood. Can cause pseudoneutropenia.
Chronic Granulomatous Disease
Rare, inherited. Abnormal oxidative metabolism in neutrophils. Results in frequent infections. Test with Nitroblue tetrazolium (NBT)
Chediak Higashi Anomaly
Rare, inherited. Usually fatal in infancy due to infection. Large, dark blue inclusions. Fusion of primary and secondary granules in neutrophils = abnormal lysosomes. Affects cell locomotion, de-granulation, and killing potential
May-Hegglin Anomaly
Rare, inherited. Large, round, pale blue inclusions. Associated with thrombocytopenia, giant platelets, bleeding.
Pyknotic Nucleus
Hyper-dense (almost black) nuclei. Indicates a cell part-way through apoptosis.
Pelger-Huet Anomaly
Benign inherited. “Pince nez” neutrophils, which function normally.
Alder-Reilly Anomaly
Large purple granules in WBC cytoplasm (looks like toxic gran but can be in lymphs). Caused by enzyme deficiency, but cells function normally.
Leukocyte Adhesion Deficiency
Rare, inherited. Absence of surface adhesion proteins on WBCs (integrins). High mortality rate from frequent infections.
Gaucher’s Disease
Lipid Storage Disorder. Deficiency of Glucocerebrosidase. Causes “tissue paper cell” with eccentric nucleus and “crinkly” looking cytoplasm
Nieman Pick Disease
Lipid Storage Disorder. Deficiency of sphingomyelinase. Causes “foamy” macrophages with tons of fat vacuoles. Often fatal by age 3.
Tay-Sachs Disease
Lipid Storage Disorder. Deficiency of B-hexoseamidase. Glycolipids and mucopolysaccharides in CNS. Often fatal by age 4.
Sea Blue Histiocyte Syndrome
Lipid Storage Disorder. Sea Blue staining of macrophages in spleen and marrow. Splenomegaly and decreased platelets. Usually benign.
Wiskott-Aldrich Syndrome
Normal B cell count, abnormal B cell function. Decreased antibody production.
DiGeorge Syndrome
Decreased T cells and Lymphoid tissue, normal B cells. Missing or dysfunctional thymus.
Dutcher Bodies
Vacuole-like inclusions in the nucleus of plasma cells, associated with multiple myeloma
Myeloproliferative Neoplasms (MPN) Symptoms and examples
- Increased RBC, WBC, PLTs
- Includes: Chronic Myelogenous Leukemia (CML), Chronic Neutrophilic Leukemia (CNL), Essential Thrombocythemia (ET), Polycythemia Vera (PV), Primary Myelofibrosis (PMF)
What mutation is most commonly associated with CML?
BCR-ABL1, aka the “Philadelphia Chromasome”, translocation between 9 and 22
CML Laboratory Presentation
- VERY high WBC count (200-500k)
- Left shift to blasts, no leukemic hiatus
- Increased Eos/Basos
- N/N Anemia
- Increased PLT
- Low LAP stain
CNL Laboratory Presentation
- Neutrophilia
- Slight left shift
- No BCR/ABL1 (Phili chromosome)
- RBCs and PLT Normal
- High LAP stain
ET Laboratory Presentation
- PLT > 1,000,000/cumm
- Abnormal appearance and function of PLT
- Increased WBCs
- JAK2 Mutation
Polycythemia Vera (PV) etiology
RBC clonal stem cell defect allows proliferation without need for EPO
PV Laboratory Presentation
- Increased RBCs (N/N, sludging)
- HGB >18g/dL
- Increased WBC and PLT
- Increased LAP
- Often a JAK2 Mutation
Relative Polycythemia
Increased HCT due to decreased plasma volume
Primary Myelofibrosis Etiology
Fibrotic tissue replaces cellular mass of the marrow
PMF Laboratory Presentation
- N/N Anemia
- nRBCs
- Teardrop cells
- Anisocytosis/poikilocytosis
- Increased WBC w/ left shift and eos/basos
- Decreased PLT
Myelofibrosis vs. Myelophithisic Anemia
- High vs low WBCs
- Fibrotic vs tumor cells in marrow