Hematopathology Flashcards
maturation delay
neutrophil hypersegmentation
Megaloblastic anemia
Pernicious Anemia
Folate Deficiency
Megaloblastic anemia
Symptoms: Neurological, poor balance, problems w/ stereognosis
Diagnosis: schiling test
Treatment: B12 therapy (GI problem- injections)
Pernicious Anemia
T/F - Humans can make folic acid.
False - Have to get folic acid through diet
Symptoms: NO neurological symptoms
Diagnosis: obtain folic acid levels
Treatment: folate therapy
Folate deficiency
What is the importance of the reticuloendothelial system (RES)?
- spleen is a major repository of mononuclear phagocytic cells
- recycling center for red blood cell constituents (iron)
- filtration of unwanted elements from the blood by phagocytosis
- major secondary organ in the immune system
- source of lymphoreticular cells and sometimes hematopoietic cells
- reserve pool and storage site of platelets, RBCs, and leukocytes
- Premature destruction of red cells
- accumulation of products of hemoglobin catabolism
- compensatory increase in erythropoiesis w/n bone marrow
Hemolytic Anemias
Manifestations: hemoglobinemia, hemoglobinuria, methmalbuminemia, jaundice, hemosiderinuria, and decrease in serum hepatoglobin
Intravascular hemolysis
Damage to red cells by mechanical, immune or toxic factors
Intravascular hemolysis
Occurs whenever red cells are injured, rendered foreign or become less deformable and are sequestered by the spleen for destruction
Extravascular hemolysis
Manifestations: Anemia, Jaundice, decrease in serum haptoglobin
Extravascular hemolysis
What are some RBC disorders associated with hemolysis?
Enzyme deficiency: glu-6-P dh deficiency
Hemoglobinopathies: sickle cell and thalassemia
most lymphoid neoplasmas are of _________ origin
B cell
T/F - all lymphoid neoplasms are moncolonal
True
Is tissue required to diagnose neoplastic proliferations of white cells?
Yes
What is the most common cancer of children?
Acute lymphoblastic leukemia/lymphoma (ALL)
What ethnicities are affected most in Acute lymphoblastic leukemia/lymphoma ALL?
3xs more common in whites than blacks
boys more common than girls
Hispanics have the highest incidence
At what age does the incidence of Acute lymphoblastic leukemia/lymphoma occur?
Age 3
The majority of all Acute lymphoblastic leukemia/lymphomas are of what cell origin?
pre-B cell origin
What is the morphology of Acute lymphoblastic leukemia/lymphoma?
Hypercellular bone marrow packed with lymphoblasts
35% blast cells in marrow or circulation
What ate the symptoms of Acute lymphoblastic leukemia/lymphoma?
anemia, fatigue, fever and infection
What is the difference between chronic lymphocytic leukemia (CLL) and small lymphocytic leukemia (SLL)?
Differ only in degree of peripheral lymphocytosis
What is the most common leukemia in adults in the western world?
Chronic lymphocytic leukemia and small lymphocytic leukemia
Tumor cells of this disease express CD19, CD20 and CD5. What disease is it?
Chronic lymphocytic leukemia and small lymphocytic leukemia
CD5 - (T cell marker only on small subset of B cells, large #s raise suspicion of malignant process)
What are two of the most common inherited disorders of bleeding ?
Hemophilia A and von Willebrand disease
What is the major source of vWF?
epithelial cells
What is the major source of factor VIII?
hepatocytes
What is the most important function of vWF?
adhesion of platelets to subendothelial collagen
What is believed to be most common bleeding disease?
vWFd
A test reveals prolonged bleeding time with normal platelet counts. What disease is it?
vWFd
A test reveals prolonged PTT with normal PT, bleeding time and platelet counts. What disease is it?
Either Hemophilia A or B
Both are clinically indistinguishable
WHat is the most common hereditary disease with serious bleeding?
Hemophilia A
Hemophilia A is a mutation of what gene?
FVIII gene
Hemophilia B is a mutation of what gene?
FIX gene
Decreased ferritin
Increased total iron binding capacity
Iron deficiency Anemia
Neutrophil hypersegmentation
Megaloblastic anemia
Neurological problems
Poor balance
Diagnosis: Schilling test
Pernicious anemia
No neurologic symptoms
Diagnosis: folic acid levels
Folic acid therapy
Hemoglobinemia Hemoglobinuria Mathemalbuminemia Jaundice Hemosiderinuria Decrease in serum haptoglobin
Intravascular hemolysis
Anemia
Jaundice
Decrease in serum haptoglobin
Extravascular hemolysis
A patient with sickle cell anemia may develop anaplastic anemia due to _______________ infection.
Parvo B19 infection
What is needed for the diagnosis for sickle cell and/or thalassemia?
Hgb electrophoresis
Characterized by a lack of or decreased synthesis of either alpha or beta globin chain of HbA
Thalassemia
Anisocytosis
RBCs are microcytic and hypochromic fragmented and stippled
Elevated reticulocyte count
Beta thalassemia major
Target cell
Bone pain due to expansion of marrow cavity
Beta thalassemia
Defect in the red cell membrane that renders erythrocytes sperical, less deformable, and vulnerable to splenic sequestration and destruction
Hereditary spherocytosis
Deficiency of spectrin - leading to reduced membrane stability and loss of membrane fragments
Hereditary spherocytosis
When red cell progenitors are normal but proliferate in response to increased levels of erythropoietin. Seen with lung disease, high altitude livingt, and cyanotic heart disease
Secondary absolute polycythemia
Test to asses platelet response
Bleeding time
What is the normal PT?
10-13 seconds
What is the normal PTT?
20-34 seconds
Adhesion of platelets to subendothelial collagen is the action of ______.
vWF
Prolonged bleeding time with prolonged PTT
vWFd
Prolonged PTT, normal PT, bleeding time and platelet counts
Hemophilia A or B
Involvement of bone marrow - blast cells
Peripheral blood
Leukemia
Most common cancer of children
3xs more common in whites than blacks
More common in boys than girls
Majority of tumors are Pre-B cell tumors
Acute lymphoblastic leukemia
Poor prognosis of child with ALL involves the presence of what mutation?
t(9;22) the philadelphia chromosome
Synthesis of kappa and lambda chains
CLL
CD5 marker - abnormal
CLL
Transformation to diffuse large B cell lymphoma so called Richter syndrome
CLL
IL6 proliferation - osteoclastic activating factor that mediates bone destruction
Multiple myeloma
Kappa light chain - bence jones proteinuria
Multiple myeloma
Translocation involving the BCR gene on chromosome 9 and the ABL gene on chromosome 22 - resultant is BCR-ABL fusion gene
chronic myelogenous leukemia - CML
A distinctive tumor giant cell known as the Reed-sternberg cells is considered to be the true neoplastic element
Hodgkin disease
