Hematology

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Anemias of Inadequate Production-Macrocytic

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megaloblastic changes (hypersegmented neutrophil): Folate, B12, inborn errors

No megaloblastic changes: Diamond-Blackfan, Pearson Marros-Pancreas, Congenital dyserythropoietic anemia

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anemia with other hematologic abnormailities

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aplastic anemia
leukemia
bone marrow replacement disorders

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anmia with hemolysis (hyperbili, increased LDH)

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Spherocyes: hereditary spherocytosis, autoimmune hemolytic anemia, Wilson disease
Sickle: SCC, Sickle beta thal
Target: Hb SC
Hypochromic: homozyg beta thal,
microangiopathy: HUS, thrombotic throbocytopenia
bite cells: G6PD

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norbmocytic anemia with low retic

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chronic diseaease
renal disease
transient erythroblastopenia of childhood
anemia with hypothyroidism

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Diamond-Blackfan Anemia

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Congenital Hypoplastic Anemia. 90% present in 1st yr. ave 3mo. macrocytic, retic low, no RBC precursors, nl bone marrow. Sporadic. defect in cell line causing too much apoptosis . High EPO in serum and urine. 1/2 have congen anomalies (triphalynx thumb, small, . Rx: steroids help 3/4

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Pearson Marrow-Pancreas Syndrome

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marrow failure anemia>neurtorpenia>throbocytopenia in neonatal period. Hb F elevated. FTT, pancreatic fibrosis, DM, muscle and neuro impariment, early death. mt DNA deletion

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Transient erythroblastopenia of Childhood

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most common acquired red cell aplasia in kids. severe, transient hypoplastic anemia 6mo-3yrs. . Transient immune suppression often post viral illness.low retic, neutropenia in 20%, platelets norml. Self-resolves.

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anemia of chronic disease

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common with cuinfection inflammation or tissue breakdown. Hb 6-9. normochromic and normocytic. retic nl to low. wbc hi. serum Fe low, TIBC nl is good indicator and diff from Fe def.

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Congenital dyserythyropoietic anemia

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Anemia despite ineffective active erythropoiesis. 3 types all singe gene.

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Folic acid

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mild megaloblastic anermia 4-7 mo before Fe deficiency. irritable, fail to gain weight, chronic diarrhea. goat's milk is deficient.

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Pernicious anemia sx

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polyendoronpathy s. adreanl, parathyroid, thryoid. megaloblastic anemia, GI, neuro

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pernicious anemia describe

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autoimmune destruction of parietal cells wthich make itrinsic factor. In kids, associated with polyendocrinopathy s.

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when to give VZIG to immunocomprimised

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within 96hrs of exposure to varicella.

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hemophilia A factor

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VIII

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hemophilia B factor

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IX

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management of headtrauma in hemophilia

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boost factor to 100% level.

Front 17

Down syndrome leukemias

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1/3 have transient leukemia, resolves spontaneously. 20% get ALL (worse than gen pub), or AML (better than gen pub)

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hereditary spherocytosis, dx, sx

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pallor, jaundice, splenomegaly, gallbladder d., often present with B19 virus, which suppresses RBC production. hemolytic anemia, hi MCHC, spherocytes (small and dense RBC with no central pallor). AD. dx by osmotic fragility test.

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hereditary elliptocytosis, dx, sx

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same as sperocytosis but elliptocytes are in smear. MCHC nl.

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G6PD deficiency, inheritance and labs.

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Xr, nl MCHC. hemolytic anemia

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Blackfan-Diamond S.

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anemia in 1st yerar. aplasia of RBC, no hemolysis. retic low, MCHC nl.

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G6PD provocatures

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bactrim, chloramphenicol, nitrofurantoin, antimalarials, DKA, aspirin,

Front 23

autoimmune hemolysis, warm Ab (types)

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most cases, idiopathic: primary
SLE, leukemia: secondary
acute: 3-6mo, ages 2-12
chronic: <2mo, >12ry mo-yrs,

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autoimmune hemolysis, warm. Rx

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prednisone 2mg/kg/day
Tx with least reactive blood may be necessary if Hb<5

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pallor, fatigue, ictuerus during or after viral illness, think

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autoimmune hemolytic anemia. direct coombs.

Front 26

Osteoid osteoma

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common benign bony tumor. sharpt boring pain. worse at night. naproxen helps. males, 5-24yrs. femur, tibia, spinke, tender radiolucent nidus, surrounding reactive bone,

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Osteochondromas

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most common benign tumor in kids. painless, hard, nontedner mass. metaphyses of long bones.

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Osteogenic sarcoma, dx

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pain, mass. bone destruction and production, periosteal elevation

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Ewing Sarcoma, dx

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pain, mass, XR: lytic, onion skin.

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Lab dx of DIC

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CBC, PT, PTT initially.

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cyclic neutropenia sx

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q21 days nadir. malaise, fever, gingivitis, oral ulcers, pharyngitis, lymphadenopathy.

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cyclic neutropenia dx

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recurrent fever, gingivitis, ulcers. ANC 2-3times week for 6-8wks.

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tumor lysis risk malignancies

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Burkitt > T-cell leukemia-lymphoma > ALL

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treatment of problematic hemangiomas

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around eye if setup for amblyopia. use prednisoneburst and slow taper.

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von Willebrand lab dx

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factor VIII clotting activity, mesaurements of von W proteins

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von Willebrand disease sx

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ecchymoses, epistaxis, menorrhagia, post op bleed. menorrhagia most common present in girls.

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thalassemia majof

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microcytic severe anemia. homozygous beta thal. Italian, Greek. almost no HbA as no beta globin. 6mo-2yr present.pallor, irritability, FTT, HSmegaly, jaundice. nucleated RBCs

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bleeding time best corelates with:

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platelet fxn.

Front 39

transient erythroblastopenia of childhood

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severe anemia and reticulocytopenia in a healthy child.toddlers. normochromic, normocytic severe. no retic response. self-limited. in recovery they retic hard, so may get jaundiced. No Rx unless CV unstable.

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Diamond-Blackfan S.

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2mo. 25% also have congenital malformations. hi fetal Hb. macrocytic. no retic. no precursors.

Front 41

difference between transient erythroblastopenia of childhood and Diamond-Blackfan.

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age. D-B presents when HbF weans off 2mo ish. Transient happens to toddlers.

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Wiskott-Aldrich

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recurrenbt infections, bleeding (thrombocytopenia), eczema. Xlinked

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Fanconi S. sx

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80% have congenital anomailes. hyperpigmentation, short stature, arm anomalies. aplastic anemia by 4yrs

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differentiating aplastic anemia and ALL

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very hard. aplastic anemia presents with palor, purpura, and all three cell lines down. ALL is 20 times more common and does not have HS megaly and bone pain. bone marrow differentiates.

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ALL relapse sites

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bone marrow moset. CNS, testes. painless testicular enlargement.

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PTT

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measures all but VII. Best measure of von Willebrand and hemophilia A. lupus anticoagulant more likely to affect PTT. more sensitive to heparin than PT. 20-35 is norm. prolonged in newborns

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PT

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measures VII, X, V, II, fibrinogen. onlyu PT gets VII. 10-13 nl. long PT with nl PTT is VII deficiency.

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factor XIII deficiency

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nl PTT, PT. delayed umbilical stump sep. elayed bruising following trauma. poor wound healing.

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Hemophilia A genetics

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X recessive. 1/3 are spontaneous. 1/2 have severe disease.

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DVT causes

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DVT in kids = hereditary thrombophilia.
factor V leiden def.
prothrombin G20210A
hyperhomocystinemia

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neonatal abdominal mass

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benign GU lesions. obstructive hydronephrosis, multicystic dyspoastic kidney.

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Wilms sx

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most common abdominal mass in kids. asx. but some have HTN, microscopic hematuria. flank mass, painless.

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sickle cell d. hepatic and bil tract compications

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cholelithiasis, liver infarction, gallstones in 40% of teens.

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autoimmune hemolytic anemia sx

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palor, fatigue, jaundice, abdominal pain, dark urine, following viral illness.

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neuroblastoma prognosis

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age and stage. <1yr great. >1yr depends on stage.

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onset of sickle cell

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hemolytic anemia starts 2-4mo/ first sx around 5-6 mo with hand-foot s. (symmetric swelling, painful of hands and feet)

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Hand-foot syndrome in sickle cell. what is it

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crowding of bone marrow chokes off blood supply. necrosis of small distal bones.

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acute chest s

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pulmonary infarction with pneumonitis or microscopic fat emboli (from bone marrow infarct).

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CVA in sickle cell

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embolic events10% of all sickle patients.

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acute splenic sequestration

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episodic event in infants and young children with SCC after acute febrile illness. Large amount of blood pools in spleen. Sudden massive enlargement, signs of circulatory collapse rapidly develop. Tx needed acutely.

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Infections of SCC

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pneumococcus, H flu infections. And Salmonella osteo. Spleen may be big, but it does not work

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B19 virus and SCC

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just like chronic hemolytic anemics. acute life-threatening decrease in Hb level with aplastic crisis.

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CV disease in SCC

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cardiomegaly by teen

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GI disease in SCC

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chronic high Fe hurts liver, gallstones in teens

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HbA

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mafor Hb of adult (a=adult)
2 alpha, 2 beta

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Hb F

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alpha 2, gamma 2. so beta thall not seen

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Hb A2

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minor adult Hb , alpha 2, delta 2

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Hb Barts

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tetramer of gamma, gamma 4

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Hb H

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tetramer of beta, beta 4. seen in alpha thal

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Hb AS

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sickle trait. one S, one A. can have splenic infarct but usually Asx

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Hb C

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alpha2, beta2with Lyx change.
in AC: (heterozygote) target cells but no anemia.
in CC: hemolytic anemia, , no sickling.
in SC: less severe than SS but similar. spindle-shaped RBCs.

Front 72

Thalassemias

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all cause microcytic anemia. all are from decreased transcription of alpha or beta. + indicates decreased transcription. 0 indicates no transcription. Only most severe alpha thal has abnormal hb (tetramers of beta or gamma.

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B-Thal major

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6m0-1yr presnts has severe progressive hemolytic anemia.transfusion dependent. untreated causes bones become thina nd fx occur. face and skull expand. serial tx to keep Hb>10. Hemosiderosis and splenectomy eventually occur in all.

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alpha thal

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a-/aa: silent carrier
a-/a-: trait. milkd microcytic anemia. Hb Barts in newborn up to 1 mo
a-/--: intermedia, Hb H disease (tetra-beta forms). Hb Barts in newborn and Hb H.micorytosis
--/--: hydrops

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alpha thal in genes in different pools

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Asians: -,-
Africans: -,a
so Asians can get Hb H and hydrops, usually not in Africans.

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neonatal hemochromatosis

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acquired s from severe liver disease, liver dysfunction, massive Fe stores

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transfusion induced hemosiderosis, Rx

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each 500cc of PRBC = 200mg Fe. deferoxamine chelates. SQ pump 5-6 times a wk

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ITP sx

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1-4yr old suddden petechiae and purpura with profound thrombocytopenia. viral infection 1-4weeks ago.

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ITP DDx

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severe isolated thrombocytopenia in healthy child has small diff. if anemia present do Coombs to rule out Evans S. (autoimmune hemolytic anemia with thrombocytopenia.

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ITP course

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up to 6 months of thrombocytopenia. 70-80% resolve by then. no problems as long as no significant bleed, including intracranial. No therapy demonstrated to improve short or long term complications

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neonatal alloimmune thrombocytopenic purpura

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Rh disease of platelets. healthy child who gest petechia in first few days. moms platelet count nl. Can occur in first born. worse in second. treat with maternal platelet tx.