Hematology Flashcards
How is hemophilia a managed?
Infusions of recombinant factor prophylactically or when bleed occurs depending on the child
What causes iron deficiency anemia in infancy, toddlers, and adolescents?
an infancy it is caused by an inadequate intake of iron. In toddlers it is often due to increased reliance on whole milk. And in adolescence it is related to dieting practices.
What is a hereditary disorder that is characterized by abnormal synthesis of alpha (4) and beta (2) globin chains?
Thalassemia
What measures the flatness or fullness or shape of red blood cells?
Red cell distribution with or RCDW
Differentiate between a sickle cell trait versus sickle cell disease?
In sickle cell trait a patient is heterozygous for hemoglobin AS. in sickle cell disease the patient is homozygous for hemoglobin SS
How are different anemia’s classified?
MCV and MCHC
What are some signs or symptoms of iron deficiency anemia?
Pica, paleness, tachycardia, lethargy
What is the MCHC?
Expression of the average hemoglobin concentration in red blood cells
What are some physical exam findings of sickle cell disease or anemia?
Jaundice, retinopathy, cardiomegaly, hepatosplenomegaly
What laboratory finding measures the size and volume of the red blood cell?
Mean corpuscular volume or MCV
What are common sources of lead?
Paint and paint dust, contaminated soil
What measures the color of red blood cells?
Mean corpuscular hemoglobin concentration or MCHC
How would you classify anemia of ACD, or acute blood loss?
Normocytic and normal chromic
What is an autosomal recessive disorder in which hemoglobin s develops instead of hemoglobin A?
Sickle cell disease
What is the CDC’s definition level of lead poisoning?
Greater than 5 micrograms per deciliter
What supplement do patients with civil cell anemia receive?
Folic acid
What is the classic symptom of cicil cell anemia?
Pain due to a vaso occlusive crisis usually in the back chest abdomen and long bones
What is the word for red cell size variation?
Anisocytosis
Why is it thought that sickle cell developed and became prominent in African American communities?
Sickled cells cannot carry malaria therefore people with sickle cell survived in areas with a lot of malaria
How does lead poisoning typically occur?
Ingestion or inhalation
If a child is anemic but the reticulocyte count is normal what does this demonstrate?
Bone marrow marrow failure, hemorrhage, or hemolysis
what is a group of malignant hematological diseases in which normal bone marrow elements are replaced by abnormal, poorly differentiated lymphocytes known as blast cells?
Leukemia
What will the bone marrow of someone with leukemia show?
Poorly differentiated blast cells that have been replacing healthy bone marrow tissue
What kind of genetic disorder is thalassemia?
autossomal recessive
What value demonstrates normochromic red blood cells?
32 to 36%
What is the normal level for the reticulocyte count?
1 to 2%
When might a person with sickle cell trait become symptomatic?
When hypoxic, in high altitude, or extreme exertion
What malignant cells can be seen on a peripheral smear of someone with leukemia?
Blast
What is hydroxyurea used for and what is the dose?
Stimulates fetal hemoglobin which cannot sickle, the dose is 35 mg per kilogram per day
What are typical laboratory findings of thalassemia?
Decreased MCV and hypochromic RBCs, and abnormal hemoglobin electrophoresis
What are the values for a normocytic MCV?
About 80 to 100
What are the key pieces of sickle cell anemia treatment?
Hydration, oxidation, analgesics, antibiotics, transfusions
What are four laboratory findings associated with iron deficiency anemia?
Low MCV, low MCHC, low RBCs, increased red blood cell distribution width
What are signs and symptoms of lead poisoning?
Vague GI symptoms, lethargy, headaches, bertonian lines that are bluish discoloration of the gingival border
At what level is chelation therapy recommended?
45 to 69 micrograms per deciliter
How is thalassemia typically diagnosed?
Newborn screening
How would you classify anemia’s of IDA, thalassemia, and lead poisoning?
Microcytic and hypochromic
What is the initial treatment of iron deficiency anemia? What is the next step in treating iron deficiency anemia?
3 to 6 mg per kilogram per day until hemoglobin normalizes. then it to replace iron stores 2 to 3 mg per kilogram per day for 4 more months
How does the RCDW change in iron deficiency anemia?
The RCDW is increased as the red blood cell is empty of hemoglobin and iron so it is flatter and wider
What is the peak instance of ALL?
4 years of age
What led level requires hospitalization?
Greater than 70 micrograms per deciliter
What level of blood poisoning needs to be referred to hematologist?
10 to 14 micrograms per deciliter
What factor is affected in hemophilia A?
Factor eight
Why are the WBCs and platelets also sometimes elevated in sickle cell anemia?
In an attempt to fix anemia bone marrow does not differentiate and increases other cell lines as well
What happens to the reticulocyte count in patients with sickle cell disease?
Increasing to 10 to 25% as immature blood cells are released in response to anemia
What is the management of thalassemia?
Referral to a hematologist
What are predisposing factors to sickle cell crises?
Infection, physical or emotional stress, blood loss
What type of genetic disorder is hemophilia A?
X-linked recessive disorder
What are common signs or symptoms of leukemia?
Anemia, pale, petechiae, epistaxis, bone and joint pain
What is the reticulocyte count?
The number of baby red blood cells or young RBCs in circulation
What disease presents with purple and petechiae or first raja, abdominal pain, histopathology with IGA, renal involvement?
HSP
What renal signs are seen in HSP?
Hematuria or proteinuria
How long does it take for ITP to resolve?
6 months
If platelets with ITP are less than 20,000 what treatment is needed?
Steroids and/or IVIG
What is von willebrand treated with?
Recombinant factor as well as DDAVP
