Hematology Flashcards by R A

Q

physiologic nadir in term infants

A

6-10 weeks low hemoglobin (9-11mg/dl)

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Q

physiologic nadir in preterm infants

A

4-8 weeks (7-9mg/dl)

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Q

When do we give Rhogam to mothers

A

administered to all Rh negative mothers at risk for Rh alloimmunization

IM injection of 300ug (1ml) human anti-D globulin within 72hours delivery of Rh positive

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Q

What is neonatal polycythemia?

A

Full term with hgb >/= 22mg/dl or Hct >65%
measure central hemoglobin
Hct peaks during 1st 2-3 weeks of life

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Q

Manifestations of early onset Vitamin K deficiency

A

bleeding in 1st 24 hours
mother chronically given anticoagulants, anticonvulsant, cholesterol lowering
can have severe bleeding

*should be given 1-2mg Vitamin K IV
if still prolonged –> 10-15ml/kg Fresh frozen plasma

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Q

Responses to iron therapy in IDA:

replacement of intracellular iron, decrease irritability, increase appetite, increase serum iron

A

12-24hr

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Q

Responses to iron therapy in IDA:

initial BM response; erythroid hyperplasia

A

36-48hr

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Q

Responses to iron therapy in IDA:

reticulocytosis peaking at 5-7 days

A

48-72hr

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Q

Responses to iron therapy in IDA:

increased hemoglobin level, inc MCV, inc ferritin

A

4-30 days

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Q

Responses to iron therapy in IDA:

repletion of stores

A

1-3 months

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Q

normal development switch from fetal to adult hemoglobin synthesis at birth that results to

A

replacement of high-oxygen affinity fetal hemoglobin with lower affinity adult hemoglobin capable of delivering more oxygen to tissues

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Q

physiologic anemia of infancy

A

inc blood O2 content and delivery –> downregulation of EPO production –> suppression of erythropoiesis –> aged RBCs that are removed from circulation are not replaced –> Hgb level decreases

point is reached between 8-12 weeks when Hgb is 11g/dL

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Q

what is physiologic anemia of prematurity?

A

Hgb decline is more extreme and rapid
min Hgb level 7-9g/dL commonly reached by 3-6 wk of age
blood loss from repeated phlebotomies
premature infant’s rbc life span 40-60 days
plasma EPO levels are lower than would be expected

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Q

RBC life span in premature

A

40-60 days

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Q

half life of transfused RBC in early preterm infants (<1250g)

A

30 days

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Q

most widespread and common nutritional disorder in the world

A

Iron deficiency anemia

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Q

dietary intake of Iron should be

A

8-10mg iron daily

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Q

infections that contribute to IDA

A

hookworm, trichiuris trichiura, plasmodium and H. pylori

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Q

desire to ingest ice

A

pagophagia

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Q

desire to ingest non-nutritive substances

A

Pica

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Q

what happens when iron stores are depleted

A

tissue iron stores depleted
reduced serum ferritin
serum iron binding capacity (serum transferrin) increases
transferrin saturation falls below normal

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Q

findings in IDA

A

reduced ferritin
reduced serum iron
increased TIBC

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Q

routine screening using hemoglobin or hematocrit is done

A

at 12 months of age or earlier if at 4 months the child is assessed to be at risk for iron deficiency

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Q

in mild anemia, when do we repeat cbc after treatment?

A

4 weeks after initiating therapy

1-2g/dL rise in hgb
iron medication should be continued for 8 weeks

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disorders of globin chain production

Thalassemia

absence of B globin production

Beta thalassemia

absence or partial reduction in alpha globin

Alpha thalasemia

symtoms of thalassemia major

profound weakness and cardiac decompensation during the 2nd 6 months of life thalassemic facies (maxilla hyperplasia, flat nasal bridge, frontal bossing, pathologic bone fractures, marked hepatosplenomegaly, cachexia spleen may be enlarged

deletion of 1 alpha globin

alpha thalassemia silent trait

deletion of 2 alpha globin

alpha thalassemia trait *microcytic anemia

deletion of 3 alpha globin

Hemoglobin H disease *marked microcytosis, anemia, mild splenomegaly and occasionaly scleral icterus or cholelithiasis

deletion of 4 alpha globin

Hydrops fetalis

treatment for alpha thalassemia

folate acid supplementation possible splenectomy intermittent transfusion chronic transfusion therapy

most common manifestation of G6PD deficiency

neonatal jaundice episodic acute hemolytic anemia induced by infections, certain drugs, fava beans *hemolysis ensues about 24-48hr after ingestion

most common and serious congenital coagulation factor deficiences

``` Hemophilia A (Factor VIII def) Hemophilia B (Factor IX def) ```

hallmark of hemophilic bleeding

hemarthrosis

what to request for possible Factor 8 or 9 deficiency

PTT *specific assays to confirm diagnosis of hemophilia

most common cause of acute onset thrombocytopenia

ITP

most common viruses associated with ITP

EBV and HIV

indications for bone marrow aspiration in ITP

abnormal WBC unexplained anemia history and PE suggestive of bone marrow failure or malignancy

management of ITP

IVIG induces rapid rise in platelet count | Prednisone 4mg/kg/24hr

role of splenectomy in patients with ITP should be reserved for 1 of 2 circumstances

1. older child (>4yrs) with severe ITP that lasted more than 1 year (chronic ITP) and symptoms not easily controlled with therapy 2. life threatening hemorrhage complicating acute ITP, if PC cannot be corrected rapidly with transfusion

decreased EPO production by diseased kidneys

Anemia of renal disease tx: oral iron therapy in all pedia pxs with CKD w/ anemia IV iron therapy- receiving dialysis

mainstay treatment in ESRD

erythrocyte stimulating agents (ESA) Darbepoietin- synthetic form of EPO

mucosal bleeding, epistaxis, easy bruising, menorrhagia

Von Willebrand disease

what are the types of Von Willebrand disease

Type I and Type II - mild-moderate | Type III - severe

type of VWD: most common type with mucosal bleeding, epistaxis, menorrhagia

Type I VWD Type I: desmopressin

type of VWD: most severe form; symptoms of mucosal bleeding, epistaxis, menorrhagia but with joint bleed, CNS hemorrhage

Type II VWD

findings in Von Willebrand

PTT prolonged

Low hemoglobin, low hematocrit with high RDA

IDA

Low hgb, low hct, normal RDW | Normal or high serum ferritin

Thalassemia

Low MCV

Iron deficiency anemia Thalassemia Sideroblastic anemia Anemia of chronic disease

High MCV

Vitamin B12/Folate Immune hemolytic Diamond-Blackfan

Reticulocyte count <3%

``` Infection Drugs Lead poisoning Acute blood loss Anemia of chronic disease Renal disease ```

Reticulocyte count >3%

Are there clinical signs of hemolysis? ``` If yes: Hereditary spherocytosis Hereditary elliptocytosis G6PD Sickle cell disease HUS/TTP Mechanical heart valve ``` If no: Hemorrhage

CBC findings in IDA

Low RBC, Low MCV, low Reticulocyte count | Increased RDW

When can we repeat CBC after starting iron therapy?

4 weeks after iron therapy; usually risen by atleast 1-2g/dL and often within normal levels

CBC findings in G6PD

low hemoglobin Heinz bodies anisopoikilocytosis bite cells

definitive diagnosis of Thalassemia

hemoglobin electrophoresis

microcytic hypochromic RBCs Target cells Heinz bodies

Thalassemia

most common cause of hemolytic anemia due to a red cell membrane defect

Hereditary spherocytosis

test for Hereditary spherocytosis

Osmotic fragility test: | confirms presence of fragile sphere-shaped RBCs

management for Hereditary spherocytosis

splenectomy is curative and recommended for: transfusion dependent patients severe disease moderate disease with frequent hypoplastic or aplastic crises, poor growth or cardiomegaly Folate supplementation

clinical hallmarks of sickle cell disease

vasooclusive phenomena and hemolysis

Howell Jolly bodies is seen in

sickle cell disease

management for sickle cell disease

analgesia and hydration Hydroxyurea to promote production of fetal hemoglobin vaccination against encapsulated organisms Folate supplementation

normal PT normal PTT prolonged bleeding time decreased platelet count

ITP

normal PT prolonged PTT normal bleeding time normal platelet count

Hemophilia

normal PT prolonged PTT prolonged bleeding time Normal or decreased platelet count

vWD

prolonged PT prolonged PTT prolonged bleeding time decreased platelet count

DIC

prolonged PT Normal or prolonged PTT normal bleeding time normal platelet count

Vitamin K deficiency

bruising and petechiae

mild ITP

more severe skin and mucosal lesions, troublesome epistaxis, meorrhagia

moderate ITP

bleeding episodes requiring transfusion or hospitalization

severe ITP

splenectomy indicated

life-threatening hemorrhage (intracranial bleed) children >/=4 years old with chronic ITP lasting >1yr children whose symptoms are difficult to control

Five cardinal symptoms of TTP

FAT RN ``` Fever Anemia Thrombocytopenia Renal dysfunction Nervous system changes ```

sex-linked hematologic disorder occurring most exclusively in males; marked by delayed clotting of blood

Hemophilia

first sign of early joint hemorrhage

warm, tingling sensation in the joint; | easy bruising

hallmark of hemophilia

hemarthrosis most common earliest joint involved: ankle older children and adolescents: knees and elbows

common manifestations of ALL

signs of marrow failure (anemia, bleeding, purpuric/petechial lesions, low grade fever) signs of infiltration (bone pain, lymphadenopathy, hepatosplenomegaly)

tumor lysis syndrome

hyperphosphatemia hypocalcemia hyperuricemia hyperkalemia

painless lymphadenopathy mediastinal mass presence of B symptoms

Hodgkin lymphoma

most common solid tumor in children

Brain tumors

most common malignant brain tumor in childhood

Medulloblastoma

treatment for osteosarcoma

chemotherapy and surgery

treatment for Ewing sarcoma

chemotherapy radiation surgery

abdominal mass that crosses midline

Neuroblastoma

abdominal pain, proptosis, periorbital ecchymoses (raccoon eyes), Horner syndrome

Neuroblastoma

painless abdominal enlargement with flank mass that does not cross the midline

Wilms Tumor *hematuria and hypertension

most sensitive test for IDA

serum ferritin

most common population group assoicated with priapism

males with sickle cell anemia

most common disease involving hexose monophosphate pathway

G6PD deficiency

most common cause of drug-immune hemolytic anemia

Cephalosporins

most common inherited abnormality of the RBC membrane

hereditary spherocytosis