Hemepathology Flashcards
How does erythropoietin promote the survival of early stem cell progenitors?
By inhibiting apoptosis
What is a major contributor causing decreased folate intake?
Poor diets and commonly alcoholism
What causes pernicious anemia?
AutoAbs to gastric parietal cells and intrinsic factor
What conditions do autoAbs of gastric parietal cells lead to?
Atrophic gastritis and achlorhydria
What does the peripheral blood smear show in megaloblastic anemia?
Macrocytosis and hypersegmented neutrophils
What is megaloblastic maturation characterized by?
Cellular enlargement and asynchronous maturation of nucleus and cytoplasm in bone marrow precursors of all lineages
What is aplastic anemia?
Disorder of pluripotent SCs that leads to BM failure
In aplastic anemia, what is the general shift of cellular components in the body?
Thrombocytopenia, anemia, leukopenia (primary granulocytopenia) with a shift towards lymphocytes and plasma cells. Erythroid, myeloid, and megakaryocytes lineages decrease.
Describe the RBCs in iron deficiency anemia
Hypochromic, microcytic
Thin woman with menorrhagia - what is the likely anemia Dx?
Iron deficiency microcytic anemia
A woman comes in with microcytic anemia and a chronic upper abdominal pain. Why can it be concluded that she is having improper heme synthesis leading to her anemia?
She is not getting adequate iron from her diet/she is bleeding into her stomach and using up her iron stores. Bottom line, her iron stores are low and she can’t make good heme.
What is the main culprit in hereditary spherocytosis?
Genetic inheritance that results in a poor RBC cytoskeleton (lack of proteins: spectrin, protein 4.2, band 3
What test is abnormal in hereditary spherocytosis?
Osmotic fragility test
What is a common complication of those with hereditary spherocytosis? Mechanism?
Cholelithiasis; Extravascular hemolysis of the sphereocytes occurs causing hyperbilirubinemia. The increased bilirubin causes formation of gallstones
Describe appearance of gallstones composed of bilirubin
Pigmented
What is hereditary elliptocytosis? What proteins are involved in this disorder?
Heterogenous group of inherited disorders involving the erythrocyte cytoskeleton featuring a horizontal abnormality with the cytoskeleton. Spectrin (self assembly), spectrin-ankyrin binding, protein 4.1, and glycophorin C.
What would be a tip regarding the hemoglobin that a nearly year-old child had beta-Thalassemia?
The child only has feta hemoglobin; His blood smear shows hypochromic/microcytic RBCs with anisocytosis (unequal size RBCs), poiklocytosis (abnormal shaped RBCs) and target cells
Describe the mechanism of splenomegaly in a patient with Beta-Thalassemia
The patient is anemic and therefore there is bone marrow hyperplasia in an attempt to pump out more RBCs. Extramedullary erythropoiesis spreads to the spleen resulting in splenomegaly.
If a patient presented with anemia and when tested had a mild increase in hemoglobin A2, what condition would you suspect?
Heterozygous Beta-Thalassemia
A mother who has a history of several miscarriages delivers a 36 week neonate who is pale and anemic. What is the likely cause of the anemia and what do you expect to see on the blood smear?
The mother likely has an IgG to an antigen of the fetus and causes compliment-mediated lysis of the neonates RBCs; On blood smear, you would expect to see immature erythroid precursors containing nuclei.
What lab results would you expect in a patient with polycythemia vera?
High Hb, High Hct, Hypercellular bone marrow, Depletion of marrow iron stores
What condition is a person with Polycythemia vera at risk for?
Cerebrovascular accident because of the increased viscosity of the blood
Mnemonic to remember what kind of sickle cell condition a person with HbS has:
The “S” stands for sickle - sickle cell disease
What is the cause of broad systemic pain in a person with sickle cell disease?
Abundant ischemia of the uvasculature results in pain in chest, abdomen and bones
What is a common complication following an autosplenectomy?
Susceptibility to infections by encapsulated bacteria (especially S. pneumoniae).
What virus causes aplastic crisis in patients with sickle cell disease?
Parvovirus B19
Describe pathogenesis of chronic disease leading to anemia
The body sequesters iron in the bone marrow to keep it from bacteria
What is acanthocytosis and what condition causes it?
Defect within the lipid bilayer of an RBC resulting in spiny projections from the surface. It is caused by chronic liver failure
What are typical cellular presentations of myelodysplastic syndrome?
Anemia, throbocytopenia, and neutropenia. Furthermore, ringed sideroblasts are also observed.
What is the Prussian blue stain used to observe?
Iron-laden sideroblasts
What is the cause for myelodysplastic syndrome?
Clonal stem cell defect
What is Fanconi Anemia?
Genetic defect that results in an inability to repair DNA and often results in acute myeloid leukemia
What is the Schilling test?
Test for VitB12 absorption
What disease are Heinz bodies indicative of?
G6PD Deficiency
What is the cause for myelodysplastic syndrome?
Clonal stem cell defect
What is Fanconi Anemia?
Genetic defect that results in an inability to repair DNA and often results in acute myeloid leukemia
What is the Schilling test?
Test for VitB12 absorption
What disease are Heinz bodies indicative of?
G6PD Deficiency
It what cancer are Auer Rods seen?
Acute promyelocytic leukemia
What do promyelocytes eventually become?
Neutrophils
What is the translocation that occurs in APL?
t(15;17); PML(15) and RAR (17) fuse
In what malignancy are smudge cells seen on peripheral blood smear?
Chronic lymphoblastic leukemia
In what population do 75% of B-ALL cases occur?
Children < 6 yo
In what disease are owl-eye cells seen?
Hodgkin lymphoma
Which type of Hodgkin Lymphoma is most common?
Nodular sclerosis
What is the most common and most important complication of multiple myeloma leading to death?
Amyloid nephropathy
A patient is observed to have marrow fibrosis and prominent megakaryopoiesis and granulopoiesis. What is the likely Dx?
Chronic idiopathic myelofibrosis
Genetic finding in person with follicular lymphoma?
Bcl-2 overexpression due to 14:18 transolcation
What type of lymphoma is Burkitt’s characterized as in Rubins?
Large B-Cell Lymphoma
What leukemia cells exhibit Tartrate Resistant Acid Phosphatase marker?
Hairy Cell Leukemia cells
What conditions are marginal zone lymphomas associated with?
H. pylori, Hashimoto, Sjoegrens
In what neoplastic disease are Dutcher and Russel bodies found? What are they?
Waldenstrom’s; Igs!
9 yo girl with petechiae and a platelet count of 20K had flu-like illness a week earlier and her BM showed megakaryocytosis. Platelets normal after 2 months. Dx?
Idiopathic Thrombocytopenic Purpura
What is the pentad of thrombotic thrombocytopenic purpura?
Thrombocytopenia, Microangiopathic Hemolytic Anemia, Fever, Renal Impairment, Neuro Syx
What two tests are utilized to Dx Paroxysmal Nocturnal Hemoglobinuria?
Ham acid test and sucrose test
What condition is characterized by uncontrolled proliferation of megakaryocytes?
Essential thrombocythemia
What is the normal count for platelets?
150-450K
What disease is characteristic of having myeloblasts in the peripheral blood?
Acute Myeloid Leukemia
What is suppurative lymphadenitis?
Lymph node swelling a site draining an infection
