- 90% spont remission
- PREDNISONE!!! if non-life threatening bleeding (for auto-immune)
- Splenectomy = life saving
- IV immunoglobulin (IVIG)
Tx for Idiopathic Thrombocytopenic Purpura
OFTEN AFTER VIRAL INFECTION!!!
Sx: petechiae, ecchymosis, epistaxis
Dx: Thrombocytopenia = low platelet count (t clot
WBC,Hgb, PT, aPPT = normal
Idiopathic Thrombocytopenic Purpura (ITP)
Most common bleeding d/o in kids 2-5 yo
Dx of exclusion
Idiopathic Thrombocytopenic Purpura
Immune attack of platelets -> decrease platelet life, often after VIRUS infection
Idiopathic Thrombocytopenic Purpura
Von Willebrand Dz
dec’d or impaired vWF doesn’t bind to Factor VIII -> no platelet plug
PT/aPPT/Platelets = normal
Prolonged bleeding time!!!!
Low vWF
vW Dz
Tx for vW Dz
DDAVP (type 1) (desmopressin)
vWF replacement
MOST COMMON INHERITED bleeding d/o among whites
vW Dz
Factor VIII deficiency!!!
Most common!!!
bleeding into joints (spont HEMARTHROSIS)
Hemophilia A
Hemarthrosis
bleeding into joints
prolonged aPPT
PT, bleeding time, platelets, vWV = normal (WHY???)
Hemophilia A and B
Factor IX deficiency !!!
Hemophilia B
infection/illness ->massive consumption of coagulation factors -> massive bleeding
Disseminated (wide spread) Intravascular Coagulation
prolonged aPPT/PT
low platelets
high D-Dimer
low fibrinogen (made in liver)
Disseminated Intravascular Coagulation
Disseminated
wide spread
DIC Tx
Tx underlying cause,
FFP, replace RBC, platelets
low fibrinogen =
normal platelets
prolonged PT/aPPT
= liver dz
+ Vit K deficiency
MOST COMMON cause of INHERITED thrombophilia in Caucasians
Factor V Leiden Mutations
PALPABLE PURPURA on LE (but NOT due to low platelets)
arthralgias, abd pain, renal dz
Henoch-Schonlein Pupura
NORMAL OR ELEVATED PLATELETS
deposition of IgA IMMUNE COMPLEXES
maybe high serum IgA
often elevated ASO (antistreptolysin O)
Henonch-Schonlein Purpura (HSP)
involves small vessels of skin, GI tract, kidneys
IgA IMMUNE COMPLEXES
preceding URI (upper resp inf: strep pharyngitis)
Henonch-Schonlein Purpura (HSP)
most common small vessel VASCULITIS in kids 2-7 yo
Henoch-Schonlein Purpura (HSP)
MOST COMMON CAUSE OF ANEMIA IN KIDS
Iron Deficiency Anemia
PERIPHERAL PANCYTOPENIA with hypocellular bone marrow
Acquired Aplastic Anemia
COMPLICATIONS: OVERWHELMING INFECTION, SEVERE HEMORRHAGE -> DEATH
Acquired Aplastic Anemia
HYPERSEGMENTED NUCLEI
Megaloblastic Anemia
ELEVATED RDW
Iron Deficiency Anemia
OSMOTIC FRAGILITY TEST: DETECTS INCREASE RBC HEMOLYSIS
Hereditary Spherocytosis
IRON MONITORING AND CHELATION
Thalassemia
HEINZ BODIES (denatured Hgb) in peripheral smear
G6PD Deficiency
INFARCTION CC=PAIN CHRONIC HEMOLYSIS (pallor, fatigue, jaundice, gallstones)
Sickle Cell Anemia
Complication: BACTERIAL SEPSIS in
Sickle Cell Anemia
BASOPHILIC STIPPLING
Lead poisoning
RESPONSE TO CHRONIC HYPOXEMIA
common = cyanotic congenital heart dz
Secondary Polycythemia (increased RBCs)
LOW RETICULOCYTE COUNT (BONE MARROW DEFECT)
acquired aplastic anemia (AAA)
immature/young RBCs, large size
elevated due to sickness or bleeding
reticulocytes
- Hx of PICA (ice clay dirt chalk)
- due to poor diet
Iron Deficiency Anemia
Iron Deficiency Anemia Labs
Microcytic MCV <78
Hypochromic (pale)
low RDW
low ferritin
low RDW
low Ferritin
Iron Deficiency Anemia
Cafe au lait spots
Short
micro/hydrocephaly
Congenital Aplastic Anemia (Falconi) / Bone Marrow Failure
Tx for Congenital Aplstic Anemia
Hematopoietic stem cell transplant (HSCT)
Aplasia
no bone marrow (can’t make RBCs)
low WBC, low neutrophils (neutropenia) low platelets (thrombocytopenia) low reticulocytes (bc bone marrow failed)
Acquired Aplastic Anemia (more common)
low MCV, small size RBCs
microcytic:
Iron deficiency anemia
Lead poisoning
Talassemia
high MCV, Big RBCs
macrocytic
B12 and folate (pregs)
normal MCV
chronic dz anemia
MCV measures
abg size of RBCs in blood
RDW measures
variation in avg sizes of RBCs
size of RBCs expressed as %
Hematocrit Hct
3x of HGb
Hgb
Hemoglobin [ ] in RBCs (dL0
SPherocytosys
SPleen