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35 Cards in this Set
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lecture 51 – advances in hematology
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How a modern blood count analyzer works:
Blood sample --> ... for Hemoglobin analysis, sample for ... and platelet count, sample for ... count |
lysate
RBC WBC |
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Direct measurements from the analyzer: RBC
Cells pass through the ... Resulting change in resistance produces “pulses”in the ... current. The ... of these pulses correlates with particle size/volume |
aperture
electrical height |
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Direct measurements from the analyzer: MCV and RDW
MCV = ... -Obtained directly from the peaks within the ... attainted from the RBC sample -Mathematically represented by total volume of RBC (hematocrit)/ total number of RBC RDW = ... -Calculated from the distribution of individual red cell volumes as compared to the MCV, expressed as a percentile |
Mean cell volume
histogram Red cell distribution width |
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Hemoglobin and Hematocrit (the “H&H”):
Hemoglobin -Converted to ... -Absorbance of ... source measured directly -Value then expressed as HGB concentration Hematocrit -Represents the ... volume of red cells -Calculated by the machine by multiplying the red cell count by the MCV (idealized) -Manual or ... hematocrit *Approximately 3% higher due to the incomplete packing of red cells Rough rule of 3’s (RBC – HGB – HCT) |
cyanmethemoglobin
light packed spun |
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The automated white blood cell count:
Total count done in same way as for RBCs (RBCs must first be ...!) Automated three-part differential -Based on volume determined by ... -Detects lymphocytes, granulocytes, monocytes Automated five-part differential -Utilizes ... and ... to extend the automated differential to the main 5 cell types: -Neutrophils -Eosinophils -Basophils -Lymphocytes -Monocytes |
lysed
impedance impedance light scatter |
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How a flow cytometer works:
Thin ... of cells (one cell wide) Passed through path of ... light source Deflection of light measured in two ways -... scatter – reflects cell/particle size -... scatter – reflects physical contents of cell Specific cell populations measured based on size and cell surface marker type (via staining) -Represented on “dot plots” |
stream
laser Forward Side |
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... – applications:
Antibodies raised against specific antigens -Linked to fluorescent compounds/dyes -Emitted light of specific wavelength -Measurement of that ... on the cells Applications -Lymphocyte studies (T and B cell markers) -Classification of leukemia/lymphoma -Measures of stem cell harvests -Diagnoses of unusual hematologic diseases *PNH *LAD |
Flow cytometry
antigen |
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... therapy in iron overload:
Chronic transfusion therapy is required for many patients with severe hemoglobinopathies -Sickle cell disease -Thalassemia -Dyserythropoietic anemias Often the cause of their death is iron ... -Myocardial hemosiderosis --> heart failure -Early institution of chelation therapy helps prevent/slow tissue ... accumulation |
Chelation
overload iron |
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Chelation therapy in iron overload:
Chelating therapy with ... (Desferal) -Subcutaneous infusion, 5-7 infusions/week, infusions of 12-16 hours Major problem with the treatment – compliance! Oral form of ...(Exjade) -Binds iron in the small bowel -Just recently approved in US -Long-term efficacy still being studied -Potential to greatly improve quality of life for transfusion-dependent patients |
deferoximine
deferoximine |
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graft versus leukemia effect and its importance in transplant patients:
A graft (autologous or allogenic) is used to attack the leukemia. So you want a slight ... effect in order to attack the leukemia in order to reduce the leukemic cells so that a potential ... in transplant patients with leukemia could occur. This is an ideal situation and is being tested. |
GVL
remission |
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derivation of IVIG and diseases commonly treated with IVIG:
IVIG derived from large pools of human ... (undergone EtOH fractionation for purification, chromatography for further purification, and detergent treated). It represents IgG ... of multiple specificities and is used for Post-... Ig replacement, Primary ... (any condition where B-cells don’t work), Acute ITP, Kawasaki’s Dz, and ... Dz’s (i.e. dermatomyositis, SLE, MG, MS, Guillain-Barre). |
plasma
antibodies transplant immunodeficiencies Autoimmune |
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Lecture 52 – Pediatric hematology
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Postnatal changes in erythropoiesis:
... production during the first week -EPO levels decrease in the first day of life, followed by a decrease in marrow erythroid precursors over the first week, followed by a ... in the reticulocyte count Combination of ... red cell survival, ... production and growth-related ... of the blood volume eventually leads to the nadir in hemoglobin concentration at about ... months of age known as “physiologic” anemia By about weeks 9-12, the Hb levels balance to about 11.3g/dL |
Decreased
drop decreased decreased expansion two |
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The “Physiologic Nadir”:
What is it: -A normal, transient anemia of infancy peaking at around ... weeks of age Why does it occur: -Relative to their new surroundings, babies are ... * Immediate fall in EPO levels, leading to decreased marrow erythroblasts -Shorter life span of the neonatal RBC -Growth-related expansion of the blood volume |
8
polycythemic |
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Nutrition, Growth and Cow’s Milk:
Cow’s milk is associated with iron deficiency anemia by at least two mechanisms: True ... due to cow’s milk (blood loss) -Occult GI blood loss -Associated with hypoproteinemia Preferential intake of cow’s milk (nutritional) -Lack of ...-rich foods at a time of rapid growth/increased needs Inhibition of iron uptake (small role, nutritional) -Decreased ... of iron in cow’s milk The first is blood loss, the second is nutritional. Calcium and casein have been posited to interfere with intestinal iron uptake |
allergic colitis
iron bioavailability |
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Hemoglobin values throughout childhood:
Birth – ... (13.5) 2 months – ... (9.0) 2-6 years – 12.5 (11.5) adolescent female – 14 (12) adolescent male – 14.5 (13) |
16.5
11.5 |
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Thrombocytopenia in newborns:
Increased ... -... -mediated *Maternal conditions: ITP, SLE, hyperthyroidism, drugs, preeclampsia *Neonatal alloimmune thrombocytopenia *Neonatal autoimmune thrombocytopenia -... (mostly DIC) *Hypoxia/ischemia,NEC, meconium aspiration, thrombosis, RDS, hemangiomas *Sepsis (bacterial, TORCH infections) -Unclear mechanism *Hyperbilirubinemia, phototherapy, polycythemia, Rh disease, TPN, inborn errors Decreased ... -Marrow ... *Osteopetrosis, congenital leukemia, leukemoid reactions, neuroblastoma -Marrow ... *Thrombocytopenia-absent-radius (TAR) syndrome, marrow failure syndromes ... -Hypersplenism |
destruction
Immune Consumptive production replacement aplasia Sequestration |
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Neonatal autoimmune thrombocytopenia:
Clinical features -Healthy newborn -Thrombocytopenia usually shortly after birth -Manifestations usually mild (e.g. a few petechiae) -... at several days of age -Rarely associated with intracranial hemorrhage History of maternal autoimmune disease, usually ... -Maternal platelet count is often low -Splenectomized mothers - baby still at risk Treatment -Infants with platelet counts < 50K is ... (at least 80% will respond) -Steroids and platelet transfusions are occasionally used |
Nadir
ITP IVIG |
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Neonatal alloimmune thrombocytopenia:
Platelet PlA1 antigen involved -Present in 98% of individuals If mother PlA1 negative and father PlA1 positive: -Infant PlA1 positive -Maternal anti-platelet antibody crosses placenta -Significant thrombocytopenia in the infant Very low platelet counts (usually 10K or less) despite maternal platelet count being ... (important distinguishing feature) |
normal
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Neonatal alloimmune thrombocytopenia:
Bleeding symptoms -Frequent minor bleeding manifestations (petechiae, purpura, blood in stools, hematuria) -Up to 15% develop intracranial hemorrhage -Severity of bleeding is higher than the platelet count would suggest due to impaired platelet aggregation Treatment -Emergent – irradiated ... platelets -Preferred - irradiated ... platelets *Matched random donor platelets lacking the offending antigen may be used if maternal platelets are not available *... can also be used in the absence of, or in addition to, maternal platelets, depending on severity of the bleeding |
random donor
washed maternal IVIG |
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Syndromes of decreased platelet production:
Thrombocytopenia absent radius (“TAR”) syndrome -Low platelets, radial agenesis -Autosomal recessive inheritance -Megakaryocytes absent or markedly decreased -Variable thrombocytopenia -Treatment *... (sparingly) to avoid alloimmunization *After 1 year of age, many patients exhibit a spontaneous increase in megakaryocytopoiesis and become transfusion-independent Amegakaryocytic thrombocytopenia -Like TAR syndrome but without the skeletal defects |
Platelet transfusions
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Miscellaneous causes of neonatal thrombocytopenia:
... and phototherapy -Mild thrombocytopenia in newborns -May be due to decreased platelet survival time ... -e.g. renal vein thrombosis, catheter related thrombosis -presumably due to ongoing consumption ... vessel hemangiomas or thrombosis ... disease of the newborn -Dilutional (from exchange transfusions) |
Hyperbilirubinemia
Occult vascular thrombosis Placental Hemolytic |
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lecture 53 – Infectious mononucleosis
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EBV:
Link of immunodeficiency (autoimmune) and cancer (... in particular) and also (but rare) associated with fulminant ... failure |
lymphomas
hepatic |
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Laboratory findings:
CBC -Usually ... WBC with ... predominance -Often mild decrease in platelet count -Presence of atypical ... Chemistry panels (“CMP”) -Mild/moderate ... of SGOT/SGPT *Liver toxicity from effects of infection -Often ... lactate dehydrogenase (LDH) *Marker of increased cell (B-cell) turnover EBV serologic testing -Monospot (heterophil) test -EBV antibody panel EBV DNA testing -PCR on serum |
elevated
lymphocyte lymphocytes elevation elevated |
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Reactive T-cell (“...” lymphocytes):
Morphology -Large, irregular outlines -“Flow” around adjacent red cells -Eccentric nuclei, coarse chromatin -Abundant (often basophilic) cytoplasm |
atypical
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Heterophil antibody test (...):
Basis of test -IgM antibody generated several days into illness -Agglutinates sheep and horse RBCs, hence “heterophil” -Addition of horse RBCs to patient’s serum results in RBC agglutination Timecourse of usefulness -Not positive at onset of illness -Peaks around two weeks into illness -Stays positive up to several months Advantages of test -Fast, inexpensive -In young adults the test will be positive in at least 80% of patients with EBV Limitations of test -Not accurate for ... -May not account for ... of patient’s symptoms |
monospot
preschool children (< 5 years of age) all |
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EBV ... profiles:
First antibodies detectable -Viral capsid (protein coat) antigen *IgM, then IgG *IgM peaks with symptoms, then fades *IgG peaks after IgM, persists for life -Early antigen *IgG, lasts several months Antibodies to EBV nuclear antigen (EBNA) -Seen only after several weeks -Persist for life Interpreting EBV antibody panels -Acute infection *Positive anti-VCA IgM/IgG, positive anti-EA IgG, negative EBNA -Prior infection *Positive anti-VCA IgG, positive EBNA, negative anti-EA IgG Reactivated infection *Positive anti-VCA IgG, positive anti-EA, positive EBNA |
antibody
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EBV diagnosis in immunodeficiencies:
... testing unreliable -Decreased/aberrant synthesis -Additional complication if receiving IVIG EBV-PCR testing for viral DNA -Can establish whether a patient infected or not -Cannot establish ... or ... state |
Antibody
chronic or acute |
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EBV and lymphoma:
*Not all lymphomas arise from EBV infection* EBV infection does increase lymphoma ...: -Pool of B-cells held in a proliferative state -Resulting pool of lymphocytes in which mutations may occur -Some of these mutations lead to genetic instability -Rapid accumulation of mutations leading to malignant cells ... lymphoma -EBV is considered causal in endemic Burkitt’s lymphoma -At least in part related to chronic B-cell stimulation ... lymphoma -EBV often associated with development of Hodgkin’s lymphoma -No causality noted -Chronic B-cell changes likely part of the association |
risk
Burkitt’s Hodgkin’s |
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EBV – associated lymphoproliferative disorders:
Post-transplant -Chronic immunosuppression (steroids, cyclosporin) -Exposure to EBV results in varying B-cell proliferation *Mild exaggeration of B-cell proliferation *B-cell lymphoma -Number of cases increasing with prevalence of allogeneic transplants ... -Acquired T-cell immunodeficiency -Loss of T-cell function (immune surveillance) X-linked ... syndrome ... accelerated phase |
HIV
lymphoproliferative Chediak-Higashi |
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hematologic changes associated with EBV infection:
Acquired ..., Agranulocytosis, Eosinophilia, Fibronolysis, ... (trifecta decrease in platelet, WBC, RBC counts), splenic ... due to splenomegaly, thrombocytopenia. |
Hemolytic Anemia
Pancytopenia rupture |
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the disease conditions commonly associated with EBV:
Mononucleosis, ... (i.e. Burkitt’s, Hodgkin’s, B-cell NHL), ... disorders (post-transplant setting, HIV, X-linked lymphoproliferative syndrome, Chediak-Higashi accelerated phase), Autoimmune Diseases. X-linked ... dz can also lead to hypogammaglobinemia, aplastic anemia, and B-cell NHL as sequelae conditions for those who survive EBV and have the mutation (inherited T-cell defect). |
Lymphoma
Lymphoproliferative lymphoproliferative |
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the laboratory testing for EBV and associated limitations:
(1)CBC: ... WBCs w/ predominant lympho’s, mild thrombocytopenia, atypical lympho’s present (2) Chemistry Panels (CMP): mild/moderate SGOT/SGPT ... (liver toxicity due to infection), ... LDH (increased B-cell turnover) (3) EBV Serological testing: Monospot (heterophil) test and EBV antibody panel (4) EBV DNA testing: PCR on serum (5) Heterophil Antibody Test (Monospot) (6) Limitations: EBV nuclear antigen (EBNA) only seen after several weeks and persists for life (7) Antibody testing ... due to decreased/aberrant synthesis and additional complications if patient is receiving IVIG (8) EBV-PCR testing for viral DNA can establish if a patient is infected or not, but CAN’T establish ... or ... state (9) Heterophil Antibody Test (Monospot) testing is not accurate for ... kids (<5 y/o) and may not account for ... of a patient’s symptoms |
elevated
elevation elevated unreliable chronic or acute preschool all |
