White blood cells Flashcards
what is a left shift
> 5% increase in percent of immature precursors (usually bands)
what is a leukemoid reaction
release of immature granulocytes into circulation, often associated with bacterial infections WBC can be very high > 50 associated with increased leukocyte alkaline phosphatase (absent in leukemia)
cause of x-linked agammaglobulinemia
mutation in bruton’s tyrosine kinase (BTK) gene (expressed in all stages of B cell development) - also important for myeloid maturation (assoc with neutropenia)
defect in hyper IgM syndrome?
defective class switching - increased serum IgM with deficiency of IgG, IgA, IgE - most commonly due to mutations in CD40 ligand
what infections do neutropenic patients get?
infections with enteric / endogenous bacteria (staph from skin, gram negatives from gut and urinary tract), fungal infections (candida)
genetics of kostmann syndrome
HAX1 mutation, autosomal recessive
most common mutation causing SCN
ELANE (AD)
x-linked cause of severe neutropenia
wiskott-aldrich
triad of shwachman-diamond syndrome
neutropenia metaphyseal dysplasia pancreatic insufficiency
what is WHIM syndrome
warts hypogammaglobulinemia infections myelokathexis
what causes WHIM syndrome
mutation in CXCR4 chemokine receptor - abnormal apoptosis / migration, retention of neutrophils in bone marrow
genetics of cartilage - hair - hypoplasia?
RMRP gene mutation AR inheritance
what is the risk in patients with cartilage-hair-hypoplasia
immunodeficiency (severe combined), heme malignancy (NHL most common)
what is barth syndrome
x-linked disorder associated with dilated cardiomyopathy, short stature, neutropenia
most common translocation seen in hypereosinophilia syndrome?
Fip1-like1 (FIP1L1) and platelet derived growth factor receptor alpha (PDGFRA) - results in constitutive TK activity
end organs affected by hypereosinophilia
skin lungs liver cardiac brain
treatment of hypereosinophilia syndrome with FIP1L1-PDGFRA mutation?
imatinib
treatment of unwell patient with hypereosinophilia?
high dose steroids must rule out strongyloides infection first
what types of infections do patients with CGD get?
catalase-positive (bacterial, fungal) frequently: pneumonia, abscesses, suppurative adenitis, osteo, bacteremia/fungemia
pathogenesis of CGD?
mutation in a subunit of NADPH oxidase resulting in defective oxidative burst and phagocytosis
five most common organisms that infect patients with CGD
aspergillus staph aureus burkholderia serratia nocardia
management of CGD?
antibiotic (Septra) and antifungal (itraconazole) prophylaxis interferon-gamma 3x/week as prophy if hx of severe, recurrent infections
what is this cell
Peripheral blood smear showing a normal human promyelocyte. Promyelocytes are larger than myeloblasts and myelocytes (>20 micron). The nucleus and nuclear chromatin and nucleoli resemble myeloblasts but the cardinal feature is the presence of many violet granules in the cytoplasm with either a dense or coarse pattern often obscuring other cell landmarks.
name the cell
Peripheral blood smear shows a normal human myelocyte. As the myelocyte divides and matures, the nucleus becomes smaller and irregularly round, nuclear chromatin becomes coarse and clumped, and nucleoli are sparse to absent. The nuclear to cytoplasmic ratio is reduced compared to promyelocytes. Specific granules are difficult to see on light microscopy.
name the cell
Peripheral blood smear shows a normal human metamyelocyte. The cytoplasm resembles the mature neutrophil and band forms; granules are small, fine blue-black or grey, and dispersed homogeneously throughout the cytoplasm. The nucleus is indented like a bean and the nuclear chromatin is coarse, clumped, and condensed peripherally.
name the cell
Peripheral blood smear shows a normal human band form. The nucleus is nonsegmented and elongated resembling a horseshoe shape; there are no nuclear lobulations but a constriction is visible. The nuclear chromatin is aggregated into evenly arranged clumps. The cytoplasm is like the mature neutrophil with pink staining and fine azure bluish granules.
fully functional phagocytic cell
name the cell
Peripheral blood smear shows a normal polymorphonuclear neutrophil (PMN). The nucleus is segmented into four lobes connected by thin chromatin strands. The cytoplasm is pink with fine, azure bluish granules. The predominant specific secondary granules are finely dispersed and stains the cytoplasm faintly pink.
