Anemia
A patient appears anemic after large-volume IV fluid resuscitation, despite an unchanged RBC mass (increased plasma volume).
What is relative anemia? Caused by increased plasma volume dilution the blood. Check MCV.
The first step in anemia classification, which is based on RBC size, is known as
What is MCV (microcytic, normocytic, macrocytic)?
This incidence of episodic hemolysis is often triggered by infection or fava beans. What clues on the PBS are helpful in diagnosis?
What is G-6-PD deficiency? Reduction of glutathionine leads to increased oxygen radicals. Bite cells are seen on smears.
A platelet count reaches #s below 150,000/µL.
What is thrombocytopenia?
This amino acid substitution causes sickle cell disease.
What is glutamic acid / valine / b6 ?
A hemoglobin disorder, characterized by a decreased rate of hemoglobin chain synthesis, due to the partial or complete deletion of its' loci from its chromosome.
What is a thalassemia; a quantitative deficiency of hemoglobin globin chains?
Clinical severity of globin chain abnormalities differs between a and β thalassemia depending on..
What is the # of genes affected? 2 b genes vs 4 a genes.
A patient has elevated blood counts with effective hematopoiesis and splenomegaly.
What is a myeloproliferative neoplasm (MPN)?
A patient with fatigue, pallor, and dyspnea due to reduced oxygen-carrying capacity from decreased hemoglobin.
What is anemia due to blood loss?
A patient has hypochromic, microcytic anemia with low ferritin, high transferrin saturation, and increased RDW.
What is iron deficiency anemia? ~ Microcytic anemia with pica and koilonychia ~
A patient complains of dark morning urine, the physichian suspects this aquired RBC membrane condition?
What is paroxysmal nocturnal hemoglobinuria?
Decreased expression of CD55 & CD59 leaves RBCS susceptible to compliment-mediated lysis.
What is a platelet disorder?
This condition triggers RBC sickling in low oxygen states. Name the disease state and phenotype.
What is deoxygenation-induced HbS polymerization, commonly seen in Homozygous HbSS or Sickle Cell Disease
The gene loci for hemoglobin chain production are located on which two chromosomes?
Chromosome 11: beta, delta, epsilon, and gamma
Chromosome 16: alpha and zeta
2 genes encode for 3 phenotypes: normal, minor, major.
What is β-thalassemia?
A myeloproliferative neoplasm characterized by increased RBC mass, low EPO levels, decreased iron stores, and JAK2 mutation.
What is Polycythemia Vera?
This hereditary, X-linked aplastic anemia stems from a defect in telomere maintenance.
What is Congenetal Dyskeratosis?
A mixed anemia with normal MCV but high RDW
What is combined anemia (iron def +B12 def)
Identify abnormal lab markers in MAHA.
What are abnormal levels of D-Dimers, LDH, and haptoglobin, with thrombocytopenia and schistocytes.
The first lab test to confirm true thrombocytopenia from artifactual?
What is a peripheral blood smear?
Vaso-occlusion from sickled red blood cells leads to tissue ischemia & pain. This occurs because sickle cell patients produce what?
What is nitric oxide synthase inhibitor? The symptoms of sickle cell crisis occur due to this.
This condition results when 1 of 4 alpha-globin genes is deleted (aa, a-), producing no symptoms.
What is silent carrier alpha-thalassemia?
This form presents with one defective beta gene and an asymptomatic microcytic, hypochromic anemia.
What is beta-thalassemia minor?
A myeloproliferative disorder, driven by a BCR-ABL fusion gene, produces a constitutively active tyrosine kinase and low LAP score.
What is CML due to Philadelphia chromosome?
A patient has adequate iron stores but cannot incorporate iron into hemoglobin.
What is sideroblastic anemia? Disorder stems from abnormal iron metabolism and heme synthesis. Iron deposits in erythrocyte mitochondria. Seen in MDNs and X-linked disorders.
Pernicious anemia involves the autoimmune destruction of parietal cells. The vital nutrient that becomes deficient in this pathologic process is...
What is vitamin B12 deficiency from loss of intrinsic factor?
A deficiency of this mellatoprotease enzyme leads to platelet rich microthrombi, hemolysis, and end organ ischemia.
What is TTP caused by ADAMTS13?
Deficiencies of what two things impairs platelet production, causes fatigue, and macrocytosis.
What are B12 or folate deficiencies?
This condition occurs when a patient inherits one HbS allele and one HbC allele, resulting in mild anemia with vaso-occlusive symptoms.
What is HbSC disease?
A mild microcytic anemia, genotypes (a-,a-) heterozygous or (aa,--) homozygous.
What is alpha thalassemia trait?
In both β-thalassemia minor and major, precipitation of which globin chain causes oxidative membrane damage leading to hemolysis.
What is alpha-globin chain precipitation?
The platelets of this MPN are functionally abnormal and are increased in number. Known as pseudo-von Willebrand disease.
What is essiential thrombocytopenia (ET)?
A patient with defective DNA synthesis causing asynchronous maturation.
What is megaloblastic anemia?
A patient with macrocytic anemia, hypersegmented neutrophils, and neurologic deficits.
What is vitamin B12 deficiency (megaloblastic anemia)?
Increased platelet consumption, normal coagulation studies, and macroangiopathic hemolytic anemia (schistocytes)
What is TTP?
A patient has a low platelet count but is not actively bleeding. Repeat CBC testing in citrate tube is normal.
What is pseudo-thrombocytopenia (EDTA-induced platelet clumping)?
In sickle cell trait, which of the following hemoglobins appears in combination with HbS?
What is HbA? Sickle cell trait is heterozygous HbSA
Moderate microcytic anemia genotype (a-,--) heterozygous/homozygous.
What is alpha thalassemia?
This condition indicated in a 7-month-old with severe anemia, bone deformities, and evidence of hepatosplenomegaly.
What is β-thalassemia major? Caused by absent beta globin chain production, leading to excess alpha-chain precipitation and ineffective erythropoiesis.
A patient has cytopenia, dysplasia, and ineffective hematopoiesis –without splenomegaly.
What is a myelodysplastic neoplasm (MDN)?
The release of what in chronic disease states contributes to its related normocytic or microcytic anemia?
What is Hepcidin? This APR binds with ferroportin, promoting iron sequestration within macrophages. Inflammatory cytokines further suppress EPO release.
A patient with chronic inflammation experiences a microcytic anemia with iron-laden macrophages
What is anemia of chronic disease?
HIV, TB, SLE, transplant rejection, sarcoidosis
Increased expression of factor III leads to a prolonged PT/PTT by an overactive run-away cascade (increased platelets and coag factor consumption).
What is DIC?
Platelet destruction + compensatory megakaryocyte proliferation. No physiologic signs of anemia, normal smears, and a normal coag profile, often following viral infections.
What is ITP? (immune mediated – NO hemolysis – NO schistocytes!!!)
This globin chain typically moves with HbS (principle-by nature of charge) via hemoglobin electrophoresis. It is made of 2 a and 2 γ globin chains.
What is HbF?
A newborn has moderate anemia and hemoglobin electrophoresis reveals β-4 tetramers.
What is HbH disease?
A PBS reveals target cells and reticulocytes with basophilic stippling in a severely anemic child requiring frequent transfusions?
What is beta-thalassemia major? target cells (hemoglobin disorder) and basophillic stippling from rapid erythropoeisis.
A disorder with clonal hematopoiesis but no cytopenia or overt malignancy.
What is a CHIP?
A high reticulocyte count in an anemic patient after acute blood loss would indicate?
What is a hyperproliferative marrow response? Anemia caused by hemolysis or acute blood loss rather than marrow failure.
A patient with chronic kidney disease develops anemia. The synthesis of the RBC stimulating hormone, occurs in the (renal cortex of the) kidney.
What is the anemia of CKD - erythropoietin (EPO) deficiency?
Thrombocytopenia with an abnormal PT/PTT suggests this broader issue?
What is a coagulation cascade disorder?
Name a common nosocomial trigger of immune mediated platelet destruction.
What are medications?
This drug increases HbF production and reduces sickling episodes by interfering with the polymerization of HbS.
What is hydroxyurea?
A fetus develops severe hypoxia due to γ-4 tetramers seen in alpha hydrops details.
What is failure of oxygen delivery due to HbBart/HbH, whose abnormally high oxygen affinity prevents adequate tissue oxygen release.
A palatable spleen with RBC indices showing decreased MCV, MCH, MCHC, Hct, & Hgb, with an increased RDW. Platelets are markedly elevated.
What is thalassemia? PBS demonstrates codocytes and schistocytes. Confirm a diagnosis with hemoglobin electrophoresis.
What are vitamin B12 and folate deficiency, arsenic and lead poisoning, copper deficiencies.
The RBC morphology seen on a PBS from a patient with chronic liver disease
What are macrocytes with increased MCV? Excess RBC membrane from defective cholesterol esterification.
Spherocytes are associated with what two conditions?
What are Immune hemolytic anemias or hereditary spherocytosis. Morphology results from defective membrane proteins or a degrading RBC membrane from splenic circulation. May also occur in burn patients.
The ultimate goal of secondary hemostasis is? Additionally, name the functional assay.
What is the formation of a fibrin clot?
What is the Thrombin Time?
A hospitalized patient develops thrombocytopenia 5-10 days after starting heparin.
What is HIT? Antibodies against heparin form PF4-heparin complexes in circulation, stimulating platelet activation, & thrombocytopenia.
The hemoglobin solubility test is based on what principle?
What is measuring visible turbitidty from decreased solubility of deoxygentated HbS in solution?
The RBC indices and bone marrow slides in alpha thalassemia major usually demonstrate...
What is increased RDW, decreased MCV, and BM hyperplasia?
In β-thalassemia major, this hemoglobin is markedly increased (30-100%).
What is HbF? Symptoms of beta-thalassemia worsen after 6 months of age due to decline of production in this hemoglobin chain.
The hematologic slide/stain and cellular morphology required for megakaryocytic MDNs diagnosis...
What are a bone marrow evaluation demonstrating the prescence of micro-megakaryocytes?