Pediatric Board Review Course Pediatric Hematology/Oncology Kusum Viswanathan, MD Chair, Department of Pediatrics Director, Divn of Pediatric Hematology/Oncology

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  • Slide 1
  • Pediatric Board Review Course Pediatric Hematology/Oncology Kusum Viswanathan, MD Chair, Department of Pediatrics Director, Divn of Pediatric Hematology/Oncology Brookdale Univ Hospital and Medical Center
  • Slide 2
  • Question 6 week old term infant referred for anemia. Hb 7.5, Retic 2 %. Mother O+, Baby A -, Direct Coombs + Mother O+, Baby A -, Direct Coombs + Cord blood Hb 14.2 g/dL. Cord blood Hb 14.2 g/dL. Jaundice of 15mg/dL at 48 hours of life, recd photo Rx and discharged at 5 days. Jaundice of 15mg/dL at 48 hours of life, recd photo Rx and discharged at 5 days. No complaints, pale, Bili 3.5, Direct 0.5. Blood smear shows spherocytes
  • Slide 3
  • Most likely explanation for the anemia 1. G 6 PD deficiency 2. Hereditary spherocytosis 3. Physiologic anemia 4. ABO incompatibilty 5. Rh hemolytic disease 6
  • Slide 4
  • Newborn anemia- important Hemoglobin at birth is 17 g/dl, MCV over 100. Falls to 11-12 by 6 weeks of age- nadir of physiologic anemia Anemia at birth could be : Hemorrhage, may not have had time to mount a retic response Acute hemorrhage- pallor and tachypnea Look at MCV- low MCV-suggestive of chronic feto-maternal hemorrhage Alpha Thalassemia trait. Alpha Thalassemia trait. Kleihauer-Betke- Hb F resistance to acid elution
  • Slide 5
  • The treatment of choice for alloimune neonatal thrombocytopenia is 1. random platelet transfusion 2. IVIG 3. Steroids 4. Exchange transfusion 5. Washed maternal platelets 6
  • Slide 6
  • Immune thrombocytopenia Auto-immune: Pregnant women with ITP/Hx of ITP Passive transfer of antibodies (IgG) from mother. Even when mother has a normal platelet count (Splenectomy) Nadir-few days; Platelets < 50,00 have 1% risk of ICH. IVIG to mother, Fetal platelet counts, C sec, US, IVGG to baby Allo or Iso-Immune: Normal platelet count in mother Similar to Rh disease; PL A1 antigen/ Zw-a negative mother. 97% of population is PL A1 positive Sensitization early in pregnancy Plt function defect because Anti-PL-A1 interferes w/aggregation. Severe bleeding more likely; first born affected Recovery in 2-3 weeks Mothers washed (PLA1 neg) platelets; IVIG; Ultrasound; Steroids
  • Slide 7
  • Kasabach- Merritt, TAR
  • Slide 8
  • 15 months old girl presented in ER with h/o URI, and scattered petechiae and ecchymoses over the body and lower extremities. 1.perform a bone marrow aspirate to confirm the diagnosis 2.Non-accidental injury; skeletal survey to rule out bony fractures 3.treatment with either IVIG or anti-D 4.Administer platelet transfusion Physical exam normal, no hepatosplenomegaly. WBC-14,000, Hb 12.8, Plts-5,000, Diff: Normal - Next step 6
  • Slide 9
  • Acute ITP Usually acute onset; immune mediated; post viral Peak 2-5 years of age, PE no lymphadenopathy (LN), hepatosplenomegaly. CBC- other cell lines normal, large plts on smear Treat if plt< 10,000 or wet ITP, Treat- IVIG best response, 48-72 hours; blocks Fc receptors,SE Anti-D (WInRho)- Rh+,hemolysis, quick response Steroids good response, block phagocytosis, reduces antibodies, SE, inexpensive, need BM BM- Increased megakaryocytes, otherwise normal Chronic- If >6 months, F>M, older, unpredictable prognosis
  • Slide 10
  • Petechiae, HSP
  • Slide 11
  • A 2 year old boy presents for evaluation of a chronic pruritic eruption. H/o recurrent epistaxis, otitis media, and pneumonia 1.Acrodermatitis enteropathica 2.Ataxia telangiectasia 3.Atopic dermatitis 4.Langerhans cell histiocytosis 5.Wiskott-Aldrich syndrome P/E reveals erythematous, slightly scaling patches on the trunk and in the antecubital and popliteal fossae. Petechiae too- most suggestive of 6
  • Slide 12
  • Large platelets Normal platelet 7-10 days Large platelets: ITP May Hegglin (Dohle bodies in neutrophils, Plt function normal). Bernard Soulier syndrome (AR, Plat function disorder). Small platelets: Wiskott Aldrich syndrome ( X-linked, recurrent infections, eczematoid rash, platelet dysfunction)
  • Slide 13
  • Platelet function defects Normal platelet number Abnormal aggregation Glanzmann thrombasthenia--- AR, Bleeding disorder, check h/o consanguinity afibrinogenemia Abnormal adhesion- Bernard-Soulier, VWD Ineffective Thrombopoiesis MayHegglin Anomaly (Large platelets) Disorders of Secretion: Storage pool, gray platelet syndrome Hermansky Pudlak Syndrome: AR, Decreased dense granules, In Puerto Ricans Oculocutaneous albinism
  • Slide 14
  • Thrombocytosis H - Hemorrhage, Hereditary Asplenia, Down myeloprol. I- Infections, Kawasaki, Immune: GVH, Nephrotic syndrome P- Polycythemia vera, Myeloproliferative, Essential L- Leukemia (CML) A- Anemia,- Iron, Vit E deficiency, Sideroblastic T-Tumors E- Epinephrine, Steroids L-Lymphoma, Hodgkins E- Exercise T-Trauma, Fractures S-Splenectomy
  • Slide 15
  • Anemia An 18 month old girl brought in for pallor. Normal diet and PMH. She is alert, interactive, only pallor, normal vital signs, No hepatosplenomegaly, lymph nodes or bruises. CBC- Normal WBC, Plt, Hb 4.5g/dl, MCV 74, Anemia Reduced production Increased destruction Loss What else do you want??
  • Slide 16
  • Reticulocyte count Normal/Low- reduced production Iron deficiency anemia- MCV will be low ALL (leukemia)- other findings, LN, HSM Diamond Blackfan anemia- TEC: Over 1 year of age, Pallor, transient rbc production failure, recovers, MCV and Hb F high during recovery, rbc transfusion, rbc ADA normal.
  • Slide 17
  • Normal smear
  • Slide 18
  • A 2 month old girl with a history of ventricular septal defect and horseshoe kidney presents for a health maintenance visit. Her parents are concerned that she is not feeding well, appearing to become tired soon after beginning to breastfeed. She is tachycardic and appears pale. Her hemoglobin level is 3.8 g/dL. Peripheral smear reveals macrocytic red blood cells. The bone marrow aspirate shows normal cellularity of the marrow with markedly decreased erythroid precursors. Which of the following findings is most likely to be identified during additional physical examination of this patient? 1. 1. Multiple superficial hemangiomas 2. 2. Cutis aplasia 3. 3. Bifid thumbs 4. 4. Speckled white rings in the periphery of the iris 5. 5. Posterior parietal hair whorl
  • Slide 19
  • Microcytic anemia is a characteristic laboratory abnormality of all listed diseases except 1. Iron deficiency 2. Lead poisoning 3. Sickle cell disease 4. Thalassemia trait 6
  • Slide 20
  • Microcytic anemia
  • Slide 21
  • Question A blood smear taken from a toddler shows microcytic hypochromic anemia. Iron supplementation therapy is started. When will the reticulocyte response be at maximum? a.1-2 days b.5-7 days c.14-21 days d.3-4 weeks e.about 6 weeks
  • Slide 22
  • Iron deficiency questions Low MCV, low MCHC, low retic, RDW can be normal, Low Iron, Incr TIBC, Transferrin low, Ferritin low Causes: Inadequate dietary intake Toddlers, too much milk, less solids, Breast fed need iron supplements Blood loss: Menstrual, GI tract, Meckels, Epistaxis D/D: Thalassemia trait- MCV much lower in prop to anemia Anemia of chronic disease- low Fe, low TIBC, normal /high Ferritin.
  • Slide 23
  • Question- Beta Thalassemia 3 year old comes for a routine check and found to be anemic. P/E Normal. CBC Hb 8.9, MCV 58, Iron levels are normal. Hemoglobin electrophoresis is done and shows Beta Thalassemia trait. All are true except: 1. The MCV is always low 2. Fetal hemoglobin is < 2% 3. Hemoglobin A 2 is > 3.5% 4. Does not respond to Iron 5. RDW is usually normal
  • Slide 24
  • Beta Thalassemia Minor Quantitative defect in globin chains Reduced production of Beta chains Hb electrophoresis Hb A- 2 Alpha, 2 Beta Hb F- 2 Alpha, 2 Gamma Hb A2- 2 Alpha, 2 Delta Excess Alpha combines with Gamma or Delta- Increased Hb F and A 2. Smear abnormalities significant even with MILD anemia. Anemia Low MCV, normal RDW, normal retic Smear shows aniso and poikulocytosis, target cells, microcytes, misshapen cells, basophilic stippling Hb Electrophoresis: Increased Hb A 2 and/or F. Normal iron studies, no response to iron
  • Slide 25
  • Beta Thalassemia Major No production of Beta chains- Chromosome 11 Autosomal recessive 25 % chance with each pregnancy Pre-natal testing for carriers Chorionic villous sampling for diagnosis Transfusion dependent-allows for normal development Pen Prophylaxis, Anti oxidants Splenectomy after age 5 Iron overload- inherent and transfusion Need chelators
  • Slide 26
  • Question 4 year old male with no complaints. Routine CBC showed a Hb9.8, MCV 62, RDW 12.5, Retic 0.2%, Normal Iron studies, Hemoglobin electrophoresis was normal with normal Hb A2 and Hb F. You suspect Alpha Thalassemia. All of the statements are true except: 1. It is carried on 4 alleles 2. The severe form is Hydrops fetalis 3. Hemoglobin H disease occurs when 3 alleles are affected 4. Diagnosed by newborn screening when a fast moving hemoglobin is noted. 5. Fast moving hemoglobin is made up of a hemoglobin with 4 beta chains
  • Slide 27
  • Thalassemia- Alpha Reduced Alpha chains 4 types- carried on 4 allelles. (xx/xx) One absent- Silent carrier (x-/xx) 2 absent- Alpha Thal trait (xx/- - or x-/x-) 3 absent- Hb H disease (x-/- -) Has 4 excess Beta chains) 4 absent- Hydrops fetalis (- -/- -) NB period: Exces

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