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1. Haptoglobin - ANSWER a normal plasma protein that binds and clears free
hemoglobin released into plasma, is depressed in hemolytic disorders
*Influenced by many factors
2. Hemoglobin - ANSWER Is filtered through the renal glomerulus and is
usually reabsorbed by tubular cells.
Hemoglobinuria will be present only when the capacity for reabsorption of
hemoglobin by renal tubular cells is exceeded.
In the absence of hemoglobinuria, evidence for prior intravascular hemolysis is
the presence of hemosiderin in shed renal tubular cells (positive urine
hemosiderin).
3. Sick cell anemia - ANSWER an autosomal recessive disorder in which an
abnormal hemoglobin leads to chronic hemolytic anemia with numerous clinical
consequences. (sickle shaped RBC)
4. Hemoglobin S - ANSWER *unstable - controls the rate of sickling
s/s: hypoxemia and acidosis, leading to the formation of sickled RBCs.
,*Sickled cells result in hemolysis and the release of ATP, which is converted to
adenosine. Adenosine binds to its receptor (A2B), resulting in the production of
2,3-biphosphoglycerate and the induction of more sickling, and to its receptor
(A2A) on natural killer cells, resulting in pulmonary inflammation.
5. Chronic hemolytic anemia symptoms - ANSWER jaundice, pigment (calcium
bilirubinate) gallstones, splenomegaly (early in life), and poorly healing skin ulcers
over the lower tibia
6. What provokes acute painful sickle cell episodes? - ANSWER infection,
dehydration, or hypoxia
7. Acute chest syndrome - ANSWER acute chest pain, hypoxemia, and
pulmonary infiltrates on a CXR and must be distinguished from an infectious
pneumonia.
8. Sickle cell labs - ANSWER HCT 20-30%
abnormal peripheral smear
Elevated WBCs
DX: hemoglobin electrophoresis
9. Hydroxyurea - ANSWER *Increased hemoglobin F
500-750mg daily
*reduces the frequency of painful crises in patients whose quality of life is
disrupted by frequent vaso-occlusive pain episodes (three or more per year)
,10. Crizanlizumab-tmca - ANSWER a monoclonal antibody that reduces vaso-
occlusive episodes by 50%.
It blocks P-selectin on activated endothelial cells and thus disrupts the adverse
interactions of platelets, RBCs, and leukocytes with the endothelial wall.
11. Voxelotor - ANSWER inhibits the polymerization of deoxygenated sickle
RBCs and increases the hemoglobin in SS patients age 12 years or older, and thus
can reduce transfusion needs.
12. Children with SS 2-16yrs need: - ANSWER annual transcranial ultrasounds
and, if the Doppler velocity is abnormal (200 cm/s or greater), the clinician should
strongly consider beginning transfusions to prevent stroke. Iron chelation is
needed for those on chronic transfusion therapy.
13. Aplastic Anemia - ANSWER a condition of bone marrow failure that arises
from suppression of, and/or injury to, the hematopoietic stem cell. The bone
marrow becomes hypoplastic, fails to produce mature blood cells, and
pancytopenia develops
most common cause: autoimmune suppression of hematopoiesis by a T-cell-
mediated cellular mechanism, so-called idiopathic aplastic anemia.
LABS: neutrophil count of less than 500/mcL (0.5 × 109/L), platelets less than
20,000/mcL (20 × 109/L), reticulocytes less than 1%, and bone marrow cellularity
less than 20%.
, 14. Causes of aplastic anemia - ANSWER Autoimmune: idiopathic, SLE
Congenital: defects in telomere length maintenance or DNA repair (dyskeratosis
congenita, Fanconi anemia, etc)
Chemotherapy, radiotherapy
Toxins: benzene, toluene, insecticides
Medications: chloramphenicol, gold salts, sulfonamides, phenytoin,
carbamazepine, quinacrine, tolbutamide
Post-viral hepatitis (viral agent known or unknown)
Non-hepatitis viruses (EBV, parvovirus, CMV, echovirus 3, others)
Pregnancy
Paroxysmal nocturnal hemoglobinuria
Malignancy: large granular lymphocytic leukemia (T-LGL)
15. Symptoms of aplastic anemia - ANSWER weakness, fatigue, neutropenia,
thrombocytopenia
pallor, purpura, petechiae, hepatosplenomegaly, lymphadenopathy, bone pain
Hallmark of aplastic anemia - ANSWER pancytopenia (neutropenia, anemia,
and thrombocytopenia)
Pancytopenia with a normocellular bone marrow causes - ANSWER SLE,
disseminated infection, hypersplenism, nutritional (eg, vitamin B12 or folate)
deficiency, or myelodysplasia
Bone marrow disorders - ANSWER Aplastic anemia