5 Written Questions

1. what does "CML" stand for?
2. what does "AML" and "ANLL" stand for?
3. GENERAL: One of the most common inherited genetic bleeding disorders. MOST COMMON IN MALE CHILDREN. X-LINKED D/O.
   
   CAUSE: 90% of persons with ___________ produce insifficient quantities of factor VIII, and 10% produce a defective form. Factor VIII is a coagulant protein that is stabilized by vWF, and is produced by the Liver and endothelial cells. CAN'T CLOT, CAN'T COAGULATE.
   
   PATHOPHYSIOLOGY: Absence of deficiency of Factor VIII prevents clotting from occurring. Chronic bleeding and inflammation causes joint fibrosis and contractures, resulting in major disability in the long run.
   A = def in Factor IIIV
   B = def in Factor IX
   C = def in Factor XI
   
   MOT COMMON IN JOINTS. HEMEARTHROSIS WILL DESTROY THE JOINT ARTICULATIONS.
   
   S/SX: Characteristically, bleeding occurs in the soft tissues, the GI tract, and the hip, KNEE, ELBOW and ANKLE joints. Spontaneous bleeding usually begins when a child begins to walk.
   
   DDX: Compare to vW disease, other bone marrow D/O's, LV disease
   
   LABS: Increased bleeding times, Measure Factors 8, 9, 11
   
   TX: Blood transfusion, Vaccinate for Hep. B
4. GENERAL: this disease is an abnormally high total red blood cell mass with a hematocrit greater than 50%. Primary _____________ is a neoplastic disease of the pluripotent cells of the bone marrow characterized by an absolute increase in total red blood cell mass accompanied by elevated white cell and platelet counts.
   
   CAUSE: Unknown. Increased platelets stuck in spleen.
   
   PATHOPHYSIOLOGY: Increase in red cell count, hemoglobin level, and hematocrit with increased blod volume and viscosity.
   
   S/SX: SOB, complaints of headache for no reason, Bright red hands, feet, face, and itches terribly (pruritis). THINK BLOOD ISSUES: blood becomes saturated with cell particles so there is less fluid in blood, large increase in basophils which contain histamine. Most common in 40-50yrs. Pain in blood forming areas: sternum, ribs, hips. WEIGHT LOSS.
   
   DDX: secondary polycythemia (dehydraytion), COPD.
   
   LABS: increased RBC, WBC, hematocrit and histamine levels. Thrombocytosis.
   
   Complications: high uric acid = stones and gout
   
   Prognosis: 2-5yrs
5. CAUSE: An inherited disorder from both parents in which an abnormal hemoglobin leads to chronic hemolytic anemia, pain, and organ failure. Amino acids don't exchange properly within the cell.
   
   PATHOPHYSIOLOGY: The abnormal structure of HbS results from a point in the beta chain of the hemoglobin molecule, with an abnormal substitution of a single amino acid, valine, for glutamic acid. The deoxygenated hemoglobin aggregates and polymerizes in the cytoplasm, creating a semisolid gel that changes the shape and deformability of the cell. The disc is in a deoxygenated shape.
   
   S/SX: None unless upon exertion, i.e. difficulty breathing. Infarctions caused by sluggish blood flow may cause chronic damage to the Liver, Spleen, Heart, Kidneys, Retina and Other Organs.
   
   DDX: Thalassemia (always seen in people of Mediterranean decent)
   
   LABS: DECREASED HEMOGLOBIN (main), slightly elevated bilirubin, serum electrophoresis, anemia, decreased RBC
   
   TX: Bone marrow transplant (10% morbidity), Chemotherapy (which retards bone marrow fx)

5 Matching Questions

1. Pernicious Anemia
2. 1) Acute Lymphoblastic Leukemia (aka Lymphocytic) "ALL"
   
   2) Acute Non-Lymphoblastic Leukemia (aka myeloblastic...older term) "AML" or "ANLL"
   
   3) Chronic Lymphocytic Leukemia "CLL"
   
   4) Chronic Myelocytic Leukemia "CML"
3. Leukemia (Acute and Chronic)
   p. 1105 Merck
4. Hematocrit (HTC)
5. Folate Deficiency
   p. 291

1. a CAUSE: Risks include exposure to ionizing radiation or chemicals (post A-bomb, benzene), prior treatments with certain amnioplastic drugs, exposure to Epstein-Barr virus.
   
   PATHO: malignant transformations usually occurs at the pluripotent stem cell level. Abnormal proliferation, clonal expansion, and diminished apoptosis lead to replacement of normal blood elements with malignant cells.
   
   S/SX: Manifestations are due to suppression of normal blood cell formation and organ infiltration be leukemic cells. Enlargement of Liver, Spleen and lymph nodes, with occasional Kidney and gonadal involvement.
   
   ACUTE:
   Occurs when a hematopoietic stem cell undergoes malignant transformation into a primitave, undifferentiated cell with abnormal longevity. These cells proliferate abnormally , replacing normal marrow tissue and hematopoietic cells and inducing anemia, thrombocytopenia, and granulocytopenia. They are bloodborne.
   
   CHRONIC:
   Usually presents as abnormal leukocytosis with or without cytopenia in an otherwise asymptomatic person.
2. b 4 Major Leukemias
   -these are all common leukemias
3. c This is a chronic illness caused by impaired absorption of vitamin B-12 because of a lack of intrinsic factor (IF) in gastric secretions. It occurs as a relatively common adult form of anemia that is associated with gastric atrophy and a loss of IF production and as a rare congenital autosomal recessive form in which IF production is lacking without gastric atrophy.
   
   -seen in people of N. American decent, chronic alcoholics, seen in people with gastric bypass or stomach staple surgeries.
   
   SX: Very pronounced fatigue (feeling run down)... can be tired from hours to days to all of the time, dyspnea comes and goes regardless of exertion, glossitis, brittle nails, sudden explosive diarrhea with possible weight loss, mental fog, decreased concentration and confusion, common in people of the 60yr age range, BURNING PAIN IN FEET or anywhere in body, wtach for an unsteady gait, and think nervous system manifestations. This is the FAR END of VIT B12 deficiency.
   
   DDX: LV disease, Alzheimer's, are they a chronic alcoholic?
   
   LABS: Decreased hematocrit, decreased RBC, decreased MCH
4. d - a measurement of the percentage of packed red blood cells in a given volume of blood
   
   - increased could be the result of water deprivation, excess use of diuretics, or gastrointestinal losses
5. e GENERAL: Folic acid is required for DNA synthesis and red blood cell maturation. It is found in vegetables, leafy greens, fruits, cereals, and meats. Megaloblastic anemia (enlarged red blood cells).
   
   CAUSE: Much of the vitamin is lost in cooking. Most causes are malnutrition or dietary lack, especially in the elderly or in association with alcoholism. Also common in pregnancy, as bearing women require 5-10x the normal daily amount of 50ug.
   
   PATHOPHYSIOLOGY: Dietary lack. Malabsorption of Folic Acid may be due to syndromes such as sprue or other intesinal disorders. Some drugs used to prevent seizures also interfere with folic acid absorption.
   
   S/SX: Similar to Vitamin B12 symptoms, only without neurologic sensations, FATIGUE, DIARRHEA, GENERAL MUSCLE WEAKNESS
   
   DDX: Vit B12 deficiency, Iron def anemia, hypothyroidism, cancer
   
   LABS: increased MCV and MCH, increased homocysteine, slightly increased WBC, decreased RBC, decreased folate
   
   TX: Folic Acid intake
   
   "this is a type of megaloblastic anemia"

5 Multiple Choice Questions

1. General timing of chronic leukemias
   1. Leukemia (Acute and Chronic)
      p. 1105 Merck
   2. Chronic Lymphocytic Leukemia
      
      -95% of patients are above 55yrs old
   3. -metastatic bone cancer
      -leukemia
      -lymphoma
   4. insidious onset and usually found randomly or by accident, during a routine exam or general blood test
2. -one of most common cancers d/t receiving chemo for a different type of cancer
   -chemo suppresses bone marrow fx and effects WBC's
   -no CNS or brain involvement
   -increased eosinophils and basophils (basophils = histamine)
   -70-90% good prognosis
   1. Acute Lymphoblastic Leukemia (ALL)
   2. Acute Myeloblastic Leukemia (AML) (ANLL)
   3. Chronic Lymphocytic Leukemia (CLL)
   4. Chronic Myelocytic Leukemia (CML)
3. Standard treatments for all leukemias:
   1. -chemo
      -bone marrow transplant (difficult)
      -stem cell research...
   2. Leukemia (Acute and Chronic)
      p. 1105 Merck
   3. -metastatic bone cancer
      -leukemia
      -lymphoma
   4. -infections
      -renal failure
      -fractures
4. START OF CLASS NOTES
   1. Hematocrit (HTC)
   2. START OF CLASS NOTES
   3. Stormy, sudden onset
   4. Cytokines
5. Complications for Multiple myeloma
   1. Iron Deficiency Anemia
      p. 289
   2. -metastatic bone cancer
      -leukemia
      -lymphoma
   3. -infections
      -renal failure
      -fractures
   4. von Willebrand's Disease
      p. 273

5 True/False Questions

1. Multiple myeloma → LABS:
   -anemia, decreased hematocrit, normo/normo
   -decreased thrombocytes
   -leukocytosis/increased RBC's
   -proteinuria
   -presence of BENCE JONES PROTEINURIA
   -Increased IgG and IgA
   -sheets of plasma cells on bone marrow

   True        False

2. Multiple myeloma → LABS: increased eosinophils, basophils, neutrophils

   True        False

3. -chemo is mainstay
   -stem cell replacement therapy will help but only buys you time (5-7 years) → TREATMENT for Multiple myeloma

   True        False

4. Multiple myeloma → malignant neoplasm of blood-forming tissues. Cancers of the WBC's involving bone marow, circulating WBC's and organs such as the Spleen and lymph nodes.

   True        False

5. Cytokines → chemicals released by T helper cells that stimulate B cells

   True        False