Hematology Flashcards
Mutation typically seen in patients with essential thrombocythemia
CALR
The primary defect in nearly 95% of Polycythemia vera patients
the primary defect in nearly 95% of patients is an acquired mutation in exon 14 of the tyrosine kinase JAK2 (V617F)
Test to confirm Polycythemia vera
Peripheral blood for JAK2 mutation
Renal disorders that can cause secondary polycythemia
renal cysts, cancer, renal artery stenosis, Bartter syndrome, and focal sclerosing glomerulonephritis.
mutation seen in hereditary hemochromatosis
HFE gene
mutations seen in patients with essential thrombocythemia
CALR and MPL
RPMI
Roswell Park Institute
Eosinophil secondary granules contain
toxic major basic protein (MBP), eosinophilic cationic protein (ECP), eosinophilic peroxidase & neurotoxin
Basophil Specific granules contain
heparin, histamine and other factors of inflammation.
Similar to mast cells in function.
large granular lymphocytes
Natural Killer cells (aka NK cells, large granular; innate)
Monocytes are precursors to
tissue resident macrophages and dendritic cells
Tissue resident macrophages are phagocytic cells (Mononuclear phagocytic system)
Contain azurophilic granules
Antigen presenting cells
The myeloid lineage
Erythrocytes
Platelets
Monocytic/phagocytic cells
Granulocytes
The most likely explanation if a patient presents with elevated basophil counts is
Chronic Myelogenous Leukemia (CML)
An adult human body contains how much blood?
5-8 liters of blood
Wright’s stain
a polychromatic stain composed of methylene blue and eosin dyes.
stains cellular elements in peripheral blood and bone marrow smears.
James Homer Wright devised Wright’s stain procedure in 1902 by modifying the Romanowsky stain.
Romanowsky stains include
Giemsa, Wright-Giemsa, Diss-Quik, and May-Grunwald-Giemsa.
Differences between Wrights and Wrights Giemsa stain
The key difference between Wrights and Wrights Giemsa stain is that Wright-Giemsa stain produces more intense basophilic/nuclear staining, while Wright stain achieves a more eosinophilic appearance. The protocol for both stains is identical, with the second step being replaced by the preferred stain.
Wright-Giemsa stain is commonly used for general blood cell analysis, counting immature red and white blood cells, platelet counts, and neutrophil counts. It is also frequently used to evaluate bone marrow specimens, and if the Wright-Giemsa stain is not clear enough, you can use Giemsa stain to enhance stain intensity.
RBC counts
4 to 6 x 10 to the 6/ml
WBC counts
4.5-11.5 x 10 to the 3/ml
Neutrophils 60-70 % (60)
Lymphocytes 18-42 % (30)
Monocytes 2-11 % (6)
Eosinophil 1-3% (3)
Basophils 0-2% (1)
“Central/Generative or Primary ” Lymphoid organs
Bone marrow and Thymus
Platelets
150-450 x 103/ml
bone marrow produces how much per day?
200,000,000,000 red cells per day,
10,000,000,000 white cells per day
400,000,000,000 platelets per day.
bone marrow produces how much per day?
200 billion red cells per day; 2 to 3 million per second,
10 billion white cells per day
400 billion platelets per day.
How many RBCs in 1 ml of blood
4 to 6 million
Largest cell in the human body
The ovum 1000 microns
Nerve cells can be 1 meter long
t cells can only recognize antigen when
t cells can only recognize antigen when processed and loaded onto MHC I or II molecules.
Most cases of CLL can be identified using antibody specific panel
CD5, CD19, CD20, CD23, and immunoglobulin light chains
The most common immunophenotype expression of CLL/SLL
coexpression of CD5, CD19, and CD23.
Fluorescence in situ hybridization (FISH) is a highly sensitive test used to detect chromosomal abnormalities in patients with CLL/SLL.
Cytogenetics evaluation of the peripheral blood smear with FISH for 17p. deletion, 11q. deletion, 13q. deletion and trisomy 12 is routine pretreatment evaluation of patients with CLL. Though all patients with symptomatic and advanced-stage CLL/SLL are treated similarly, patients with 17p. deletion or 11q. deletion requires special consideration.17p. deletion causes CLL by TP53 mutation. TP53 is a tumor suppressor gene which is located on the short arm of chromosome 17. The normal “wild–type” TP53 gene activation occurs in response to damaged DNA and/or other stressors such as hypoxia, leading to cellular apoptosis. The deletion/point mutation of TP53 leads to CLL. 11q chromosome contains the ataxia-telangiectasia mutated (ATM) gene.
ataxia-telangiectasia mutated (ATM) gene
ATM kinase is responsible for the delayed progression of the cell cycle in the presence of DNA damage, allowing the cell to repair the damage. ATM kinase phosphorylates tumor suppressor p53 protein in the presence of DNA damage. The phosphorylation of p53 protein ultimately leads to cell cycle arrest/apoptosis of cells with DNA damage. In the absence of ATM kinase in 11q. deletion, phosphorylation of p53 does not occur, which hence cannot prevent the cell cycle arrest in DNA damaged cells.
Richter’s transformation
About 2 to 10% of CLL patients undergo Richter’s transformation, where CLL evolved into an aggressive lymphoma, most commonly diffuse large B-cell lymphoma, which presents with fever, rapid enlargement of previously stable nodal disease, and severely rising LDH levels. CLL can also transform into high-grade non-Hodgkin’s lymphoma by involving into B-cell prolymphocytic leukemia.
