Presentation of hematologic disorders - Answers -anemia, polycythemia, neutropenia,
thrombocytopenia, coagulopathy
anemia - Answers - Pallor, fatigue, heart failure and jaundice
polycythemia - Answers - Irritability, cyanosis, ruddiness, jaundice, seizures, stroke
neutropenia - Answers - Fevers, oral ulceration, lymphadenopathy, bacteremia
thrombocytopenia - Answers - Petechiae, ecchymosis, GI hemorrhage, epistaxis
coagulopathy - Answers Bruising, hemarthrosis, mucosal bleeding
sites of erythropoiesis: yolk sac - Answers starts at 3 weeks gestation (Embryonic Hemoglobin)
sites of erythropoiesis: liver - Answers moves to this area at 2 months gestation (Fetal Hemoglobin (two
alpha and
two gama chains)
sites of erythropoiesis: bone marrow - Answers -moves from the liver to the bone Marrow at 5-6 months
gestation (Adult Hemoglobin two alpha and two beta chains).
-Therefore an extremely premature infant may have significant extramedullary hematopoiesis.
-This is also why a bone marrow examination is often critical in patients with cytopenia.
-Erythropoietin production stops shortly after birth due to the rapid increase in
oxygen saturation
physiologic Nadir - Answers -Occurs at 8 to 10 weeks of life is worse in premature infants and is due to:
-Fetal RBC shorter survival time (only 60 days)
-Rapid growth of the patient in the first few months of life
-Cessation of erythropoiesis shortly after birth until the decrease in hemoglobin
triggers a decrease in oxygen delivery and erythropoiesis resumes.
Immature neutrophils - Answers controlled by granulocyte-macrophage colony-stimulating factor (GM-
CSF)
Mature neutrophils - Answers - controlled by granulocyte colony-stimulating factor (G-CSF)
, Developmental Hematology Neutrophils - Answers -Rapid increase in neutrophil count that occurs with
infection is under control of
GM-CSF
-Mature neutrophils and bands are only cells which are fully functional for
chemotaxis, phagocytosis and bacterial killing.
-Neutrophils migrate from bone marrow into circulation for 6-7 hours and migrate to tissues where they
become end stage and do not recirculate.
Developmental Hematology Eosinophils - Answers Eosinophils are under control of interleukin 3 and are
the host defense against parasites with a prolonged survival in the host's tissues
Developmental Hematology Platelets - Answers -Platelets develop from Megakaryocytes by invagination
and budding.
-Thrombopoietin is the primary regulator of platelet production.
-Platelets adhere to damaged endothelium via the adhesive proteins- von Willebrand factor (vWF) and
fibrinogen.
-Platelets circulate for 7 to 10 days
Newborn Anemia - Answers -Physiologic Nadir- At birth hemoglobin increases for 6-12 hours starting at
16.5 to
18 mg/dl and then decreases to the Physiologic Nadir of 11 to 12 mg/dl at 3 to 6 months for a term
infants and the same number at 1 to 2 months for a preterm
infants.
-This Physiologic Nadir is not abnormal and does not result in any signs of illness and does not require
treatment.
Newborn Anemia due to Decreased RBC Production - Answers -RBC Aplasia- Diamond-Blackfan anemia
-Infection- congenital viral infections (parvovirus, rubella) acquired bacterial or viral sepsis
-Nutritional deficiencies- Protein, iron, folate, vitamin B12
-Congenital Leukemia
Newborn Anemia due to RBC destruction - Answers -hemolytic disease
-hemolytic disease of the newborn
-erythroblastosis fetalis