Flashcards in Ch. 6 - Non-Neoplastic WBC Abnormalities Deck (155):
what are the four methods to measure WBCs in the lab?
1. automated hem. analyzers2. bone marrow aspirate and biopsy3. flow cytometry4. peripheral smear
what are the three general factors that affect neutrophil concn in the blood?
1. bone marrow production and release2. rate of egress to tissue and survival time in the blood3. ratio of marginating to circulating neutrophils in peripheral blood
What is the number at which someone has neutrophilia?
ANC> 7.0 x 10^9/L
T/F: Neutrophilia can be in response to a physiologic, not just pathologic process
How long does it take for marginated neutrophils to return the to circulating pool?
Stress, steroids, epinephrine, and what cytokine can move neutrophils from margination into the ciruclating pool?
How long does it take for neutrophils from the marrow storage pool to be released into the blood?
4-5 hours; released by IL6
How long does it take to increase the marrow mitotic pool?
How does epi increase the neutrophil count?
causes them to demarginate
What are six major categories of causes of neutrophilia?
Vascular collagen and vasculitis are (blank) causes of neutrophilia
Bacteria, fungi, parasites, viruses, and spirochetes are (blank) causes of neutrophilia
stress, exercise, and pregnancy are (blank) causes of neutrophilia
Carcinoma, sarcomas, and MPDs are (blank) causes of neutrophilia
burns, ischemic necrosis, and tissue damage are (blank) causes of neutrophilia
What is the ANC in REACTIVE neutrophilia?
Reactive neutorphilia shows with a (blank) shift in myeloid maturation to band cells
Toxic granulation, Dohle bodies, and vacuolization are morphologic changes of neutrophils seen in (blank)
left shifting during reactive neutrophilia
T/F: the leukemoid reaction is benign
The ANC in the leukemoid reaction is usually (blank) and has many circulating (blank) leuokcyte precursos
>50 with many immature leukocyte precursors
What type of cell is ocassionally present during a leukemoid reaction
What disease do you need to exclude when the leukemoid reaction is on you DDx?
The (leukemoid/leukoerythroblastic) rxn has the presence of nucleated RBCS
the LEB reaction has a left shift in (blank) maturation
The (leukemoid/LEB) rxn is associated with myelophthisic processes, severe hemorrhage, hemolytic anemia, or MDS
LEB; anemias and hemorrhage make you think of RBCs
What are the limits in whites and blacks for neutropenia?
What is the ANC to define agranulocytosis?
Pseudoneutropenia is caused by (blank)
What drug that is used to treat schizophrenia can cause agranulocytosis?
T/F: Cachexia often leads to neutrophilia from the presence of a shock state
phony as a 3 dollar bill. Cachexia = neutropenia
What are the six general causes of neutropenia?
iCHOADintrinsic defectsCachexiaHematologic disordersOVERWHELMING infectionAutoimmuneDrugz
What are the 4 intrinsic defects that cause neutropenia?
F*CCK!!1. Fanconi's2. Kostmann's3. Cyclic neutropenia4. Chediak-Higashi
Megaloblastic anemia, myelodysplasia, marrow failure, hypersplennism are all (blank) disoders that lead to neutropenia
Chloramphenicol, EtOH, chemo, abx, and clozapine can all cause (neutrophilia/neutropenia)
What are the three protozoal infections that commonly cause neutropenia?
Malaria, Kala-azar, Relapsing fever
Of the intrinisic defects leading to neutropenia, what is the only one caused by a maturation defect? What is the cause of all of the others?
Chediak-Higashi is caused by a maturation defect. All the others are caused by myeloid hypoplasia
Name this disease: Panmeyloid hypoplasiaHeterogenous presentation due to csome instabilityPresents in childhood: aplastic anemia, congenital physical deformities.Susceptible to hematopoetic and solid organ malignancies
Fanconi's Anemia-- you'll see the XRay of the weird joints
Name the disease:ANC
What is the medical name for Kostmann's Syndrome?
Infantile genetic agranulocytosis
Name the disease:Presents in infancy or childhoodAuto. Dom with variable exrpression21-30 DAY PERIODICITYANC
ELA (1/2) is also known as neutrophil elastase
What are the two most common congenital neutropenias?
Pregnancy induced hypertension and infection
EDTA-dependent agglutinin, old speciments, WBC fragility and paraprotein formation can all lead to (blank) neutropenia
What is this morphology?Azurophilic granules associated with Dohle bodies and vacuolization during septicemia and other toxic states
What makes up a Dohle body?
remnants of free ribosomes and the rER
When do you see Dohle bodies?
During severe bacterial infections, pregnancy, burns, and toxic states
(Toxic granulation/Dohle bodies/Vacuolizatoin) is a precursor to sepsis
T/F: vacuolization may be a false finding in stored blood
Why do we get vacuolization in neutrophils?
Lots of dead and dying material around them gets phagocytosed, that's why its an early marker of sepsis as it indicates everything is fucking dying
Shistocyte RBC morphology indicates the pt could be in what life threatening condition?
If you see a bacteria (such as a diplococci like S. aureus) inside a neutrophil, you are looking at what morphologic state?
What stages of neutrophil maturation do we see toxic granulation?
Meta, band, and neutrophil stages
The Pelger-Huet Anomaly is autosomal (dom/rec)
dominant; has homo and heterozygous forms
T/F: Pelger-Huet anomaly indicates a functional defect in the neutrophil
false; clinically asymptomatic
A pince-nez nucleus indicates what morphologic state?
The Pelger-Huet anomaly can be confused with a reactive (blank) shift
What are the general characteristics of a Pelger-Huet anomaly?
Hyposegmented neutrophils, sparse granules, single or pince-nez nucleus
>5% of neutrophils with (blank) or more lobes or any single one with (blank) lobes constitutes hypersegmentation
5% with 5, or one with 6
What states are associated with hypersegmentation?
Megaloblastic anemia (B12 def), chronic infection, MDS
T/F: Hereditary hypersegmentation is associated with refractory megaloblastic anemia
false; associated with no disease!`
A loss of vibratory proprioception indicates what vitamin def?
The Alder-Reilly anomaly is auto (dom/rec)
Large, purplish Granules that stain METACHROMATICALLY with TOLUIDINE BLUE lets you know you're looking at what anomaly?
The anomaly that is associated with Hunter's and Hurler's Syndromes is:
Alder-Reilly"Does he REILLY have Hurlers?!"
Mucopolysaccharidoses is a disease state assoicated with what neutrophilic change?
Chediak-Higashi is autosomal (dom/rec)
Name the disease:1. Giant gray-green peroxidase- positive bodies in cytoplasm of leukocytes and other cells.2. Abnormal melanosomes (skin hypopigmentation, photophobia)3. Lymphadenopathy and hepatosplenomegaly
The giant peroxidase bodies in Chediak-Higashi are (blank) bodies
What is the defect that causes the formation of inclusion bodies in Chediak Higashi?
fusion of cytoplasmic membranes, locomotion, and chemotaxis; therefore hypopigmentation because melanosomes can't move their melanin to the skin surface!
T/F: Chediak Highashi is both a morphological and functional defect
What autosomal dominant trait has large Dohle-body like inclusions in granulocytes that contain RNA?
Which myosin heavy chain is fucked up in May-Hegglin anomaly?
myosin heavy chain 9
T/F: the dohle-body like inclusions in May Hegglin anomaly cause a functional defect in GRANULOCYTES
Describe the platelet morpholgoy in May Hegglin? What effect does this have on their function?
Giant oval platelets, associated with thrombocytopenia and bleeding disorders
What disease can't handle catalase organisms because of a defect in the respiratory burst oxidase (NADPH oxidase) system?
What are the two methods of inheritance of CGD?
sex linked and auto recessive inheritance
In CGD neutrophils form (blanks) when they phagocytize but do not kill
Defects in membrane-associated cytochrome b (subunits gp91 and p22), and cytosol-associated p47 and p67 are the molecular causes of (blank)
T/F: defects in ANY portion of the NADPH oxidase system will lead to CGD
What is the difference between CGD and myeloperoxidase deficiency?
MPO still has NADPH oxidase activity but can't make bleach aka HOCl aka hypochlorite; whereas CGD has NO NAPDH activity!
On the NBT (nitrobule tetrazolium test), MPO would be (blue/yellow-pink) while CGD would be (blue/yellow-pink)
MPO: blueCGD: yellow-pink
What is the treatment for CGD?
T/F: MPO def can be acquired or genetic
In what two cells do you get an MPO def?
neutrophils and monocytes
T/F: infections are a common complication in both CGD and MPO
false; only in CGD
How do you diagnose CGD or MPO?
CGD: NBT testMPO: MPO stain
Which LAD deficiency involves Selectins?
Which LAD deficiency involveds CD11a/CD18
Which LAD deficiency involves CD11a/CD18 activation?
Recurrent pyoderma gangrenosum or a failure of umbilical cord separation are disroders associated with which LAD deficiency?
What is the threshold for eosinophilia?
Eosinophilia is associted with (cell mediated/humoral) immunity
When would you see eosiniphilia in kids?
with allergies or GI food allergies
What is the most common cause of eosinophilia? Be specific!
Tissue INVASIVE parasites
Addison's Disease often causes (neutrophilia/eosinophilia)
Collage disease often causes (eosinophilia/neutrophilia)
Besides parasites, what is a major general class of causes of eosinophilia?
idiopathic reasons; leukemias and GI disorders
T/F: allergies and infections can both cause eosinophilia
What is the threshold for eosinophilic leukemia?
>1.5 x 10^9/L
t/f: eosinophilic leukemia has no known cause
Describe Loffler's syndrome?
Pulmonary infiltrate with eosinophilia syndrome and tropical eosinophilia
What parasite often causes Loffler's?
Charcot-Leyden crystals from eosoniophil granule fusion are seen in what disorder?
Charcot-Leyden crystals are particularly dangerous to what organ?
heart; release of granule contents
Corticosteroids sequester Eos in the (blank) leading to (blank)
lymph nodes; eosinopenia
Sequestration of eos in the lymph nodes can be caused by the release of corticosteroids from what disease processes?
1. Cushing's syndrome2. ACTH3. Acute stress/ Epi4. Inflammation5. Prostaglandins6. Bacterial infection
What is the threshold for basophilia?
>0.2 x 10^9/L
What cancer often causes basohpilia?
Immediate hypersensitivity reactions will cause (baso/eos)ophilia
T/F: basophilopenia is a life threatening condition
false; clinically insignificant
What is the threshold for monocytosis?
monocyte counts vary with (age/gender)
T/F: unexplained monocytosis is common in cancer
What is the threshold for monocytopenia?
What body fluid in babies has a high monocyte count?
Monocytes are a marker of (acute/chronic) disease processes
Post-splenectomy pts will often shown elevated counts of what cell type?
Langerhans Cell histiocytosis, Letterer-Siwe disease are both disorders of what cell type?
(Monocytosis/penia) is a finding common in hematologic malignancy
Aplastic anemia, hairy cell leukemia, and glucocorticoid therapy are all causes of (monocytosis/penia)
Gaucher DiseaseNeimann-Pick DiseaseTay-Sachs DiseaseFabry DiseaseWolman’s DiseaseTangier Diseaseare all (blank) storage disorders of monocytes
(blank) disease ppresents with glucocerebrosidase deficiency in macrophages in the bone marrow, lymph node, liver, and spleen
In Gaucher disease, serum acid phosphatase is (up/down)
What is the threshold for lymphocytosis in adults and children?
Adults: >4.0kids: >9.0
Most changes in lymphocyte counts are due to (T/B) cells
(T/B) cells account for 60-80% of PERIPHERAL blood lymphocytes
Majority of circulating lymphoid cells are CD(4/8) T cells
Coxsackie A and B6, Echovirus, and Adenovirus are common causes of acute infectious (blank) in children
Persistent polyclonal B cell lymphocytosis should raise your suspicion of:
What two risk factors increase your chance of having a persistent polyclonal B cell lymphocytosis?
female smoker and post-splenectomy
Infectious mono infects (monocytes/B cells)
T/F: mono is self limiting
Infectious mono gains entry to the cell via what receptor?
What is the wBC range for someone with infectious mono?
What Ab is positive in people with infectious mono?
what is the threshold for lymphocytopenia in adults and kids?
What congenital immunodef can cause lymphocytopenia?
T/F: abnormal lymph circulation can lead to lymphocytopenia
T/F: AIDS or HAART can cause lymphocytopenia
Most SCID pts are (m/f)
Describe the difference in T and B cell counts in X linked vs. autosomal forms of SCID?
Xq13: absent to severely reduced T cells; thymic hypoplasiaAutosomal: severe def of both T and B cells (ADA def)
What is the Tx for SCID?
bone marrow transplant
What is the mode of inheritance of WA syndrome?
What are the three hallmakrs of WA syndrome?
eczema, thrombocytopenia, and immunodef
Pts with WA syndrome are at an increased risk of secondary (blank)
Pts with WA syndrome have a progressive decrease in (blank)-dependent immunity
T/F: WA syndrome patients have absent Abs to blood groups
Describe the mitogenic response to CD43 in WA syndrome patients?
When would a WA syndrome pt present with abnormal bleeding?
(blank) syndrome presents with abscence/hypoplasia of thymus, hypoPTH, congenital heart defects, dysmporphic facies, HYPOCALCEMIA, and is caused by a del22(q11.2)
X-linked Agammaglobulinemacauses a block in the B cell maturation at the (blank) phase due to a failure of variable and constant regions of IgM to connect
pre-B cell stage
X-linked Agammaglobulinemapresents with absent (blank) cells
plasma cells and decreased B cells
What is the Tx for X-linked Agammaglobulinema
What disease is auto. rec. and presents with Progressive ataxia, immune dysfunction, increased risk of malignancy, Defect in cell-mediated immunity with thymic hypoplasia or dysplasia?