Exam 3

• Thalassemia


  inherited defect in ability to produce hemoglobin, leading to hypochromia
• Typical RBC morphology of Beta Thalassemia

  Microcytic, Hypochromic, NRBC
• RBC indicies of Beta Thalassemia


  Decreased MCV, Hgb, Hct
• 2 major types of Thalassemias

  Alpha and Beta
• Thalassemia minor/trait

  -Heterozygous form

  -"AKA" Cooley's trait
• How many types of Alpha Thalassemia are there?

  4
• Silent Carrier


  1 alpha gene deletion

  No clinical manifestations
• Alpha thalassemia trait

  2 normal alpha genes

  2 missing alpha genes

  +/- mild anemia
• Hemoglobin H disease

  Alpha-globin chain disease in which three of the four genes are deleted
• Hemoglobin Barts

  4 alpha gene deletions

  incompatible with life
• Hemoglobin C

  
• Hemoglobin C disease


  Caused by amino acid substitution of lysine for glutamic acid in the sixth position on the beta chain(LG6B)
• toxic granulation

  
• Dohle bodies


  Round-oval pale grayish-blue inclusions that are found in the periphery of the cytoplasm of neutrophils, accompanies toxic granulation

  
• reactive lymphocyte


  Lymphocytes that have been stimulated to produce antibodies or lymphokines

  Appear to have increased cytoplasm, increased basophilia

  
• Pelger-Huet anomaly

  WBC disorder in which neutrophils, eosinophils, and basophils are not segmented properly, but function normally

  Mimics a left shift

  
• Smudge cells


  Chronic Lymphocytic Leukemia (CLL)

  
• Barr body


  A dense body formed from a deactivated X chromosome.

  
• Monocytes


  An agranular leukocyte that is able to migrate into tissues and transform into a macrophage.

  
• Chediak-Higashi


  membrane protein defect in transferring lysosomal enzymes to phagocytic vacuoles

  
• Hypersegmented neutrophils

  Megaloblastic anemia (B12 deficiency: neurologic symptoms; folate deficiency: no neurologic symptoms)

  
• May-Hegglin Anomaly

  autosomal dominant trait disorder where neutrophils have blue-staining inclusions that resemble Dohle bodies; thrombocytopenia is also present with giant abnormal platelets

  
• Alder-Reilly Anomaly

  autosomal recessive trait associated with disorders that prevent normal breakdown of mucopolysaccharides, characterized by presence of prominent, dark staining, coarse cytoplasmic granules

  
• Gaucher

  autosomal recessive

  
• pyknotic nucleus

  Thick Dark Nucleus usually found in Orthochromatophilic Erythroblast

  
• Niemann-Pick Disease

  sphingomyelinase deficiency results in macrophages that have soap-bubble appearance.

  
• Auer rods

  Acute myelogenous leukemia (especially the promyelocytic type)

  
• Leukemia


  Overproduction of blood forming cells (immature and mature) in the bone marrow and peripheral blood
• lymphoma


  malignant tumor of lymph nodes and lymph tissue
• Acute Leukemia


  -affects all ages

  -rapid onset

  -Untreated (death in weeks/months)

  -Most cells Blasts

  -decreased Plts

  -Usually elevated WBC

  -Pt usually anemic
• Chronic Leukemia

  -Affects Older people

  -Slow onset

  -Untreated (death in months/years)

  -More mature cells seen

  -plts normal to increased

  -elevated WBC

  -Pt usually anemic
• Leukemia Treatments

  -Chemotherapy (targeted chemical attack on cancerous cells)

  -Radiotherapy (radiation applied to infected site designed to destroy cancerous cells)

  -BM Transplant (Designed to eliminate defective stem cells and replace with cells that function properly)
• Treatment objectives for Leukemia

  -Cytoreduction

  -Remission

  -Palliative
• Etiology of Leukemia

  -Oncogenes

  -Ionizing radiation

  -chemical exposure

  -Genetic factors

  -Infectious agents