Hematology Flashcards
Peripheral smear findings in HUS
Schistocytes and burr cells, minimal platelets
Triad of hemolytic anemia, thrombocytopenia, and acute renal failure
HUS
Fetal hemoglobin takes over at
12 weeks gestation (gone by 12 months)
Red cell lifespan in newborn
45-60 days
Hypersegmented PMNs
Megaloblastic anemia (B12, folate)
Basophilic stippling
Lead poisoning
Howell Jolly bodies
Functional asplenia
Schistocytes
Microangiopathy
Spherocytes
Spherocytosis, AIHA
Target cells
Thalassemia, liver disease
Teardrops
MDS
Burr cells
Uremia vs. liver disease
Mentzer index less than 12
Thalassemia
Mentzer index above 12
Iron deficiency
How to calculate Mentzer index
MCV/RBC
Newborn screen only with hemoglobin F
Beta thalassemia major (tx with stem cell transplant)
Goats milk is deficient in
Folate
Vegans are deficient in
B12
Mode of inheritance for spherocytosis
AD
Normalcy tic anemia with reticulocytosis and increased MCHC
Spherocytosis
Gold standard test to diagnose spherocytosis
Osmotic fragility
Patients with spherocytosis s/p splenectomy still at risk for
Cholelithiasis
Mode of inheritance of G6PD Deficiency
X linked AR
Can cause G6PD problems
Fava beans, sulfonamides, antimalarials, moth balls
Bite cells and blister cells
G6PD Deficiency
Assay for CD59 or FLAER tests for
Paroxysmal Nocturnal Hemoglobinuria
ELA2 gene, chronically low ANC, risk of AML/MDS, G-CSF is tx of choice, AR
Kostmann’s Syndrome (severe congenital neutropenia)
Severe neutropenia, GI symptoms, AR
Shwachman Diamond Syndrome
Diagnose CGD with
NBT test
AR, oculocutaneous albinism, neutropenia, prolonged bleeding, increased infections
Chediak Higashi
IgE level over 1,000, cold skin abscesses 2/2 staph aureus, need bactrim ppx
Job Syndrome (hyper immunoglobulin E)
Pentad of TTP
MAHA, neurologic symptoms, renal dysfunction, thrombocytopenia, fever
Thrombocytopenia, absent radii, normal thumbs
TAR syndrome, AR
X linked, thrombocytopenia, severe eczema, immunodeficiency, SMALL platelets,
Wiskott Aldrich. SCT is curative
AR, thrombocytopenia with GIANT platelets
Bernard Soulier Syndrome
Normal platelet count, severe bleeding and bruising
Glanzmann’s Thrombasthenia. Tx with platelet transfusion
Large hemangioma with DIC
Kasabach Merrit
AR, no thumbs, short, anemia or thrombocytopenia, risk of AML
Fanconi Anemia. Defective DNA repair
Normal red cell adenosine deaminase, HgB 6-8.
Transient Erythroblastopenia of Childhood. Self limited. May be 2/2 viral infection. Just monitor
Short, webbed neck, heart disease, anemia, renal anomalies, hypotonia dims, glaucoma, MR. Abnormal red cell adenosine deaminase
Diamond Blackfan Anemia. SCT for steroid refractory pts
Lyric punched out lesion on skull, external otitis that does not respond to antibiotics (draining ears). May have floating teeth or bad dermatitis
Langerhan’s Cell Histiocytosis
Hyperlipidemia, hyperferritinemia, fever, HSM, CNS symptoms
HLH (Hemophagocytic Lymphohistiocytosis). Tx with etoposide
Mode of inheritance of Hemophilia A
X linked recessive
