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Section 10 — Hematology & Jaundice Pending expert review v1.0 · July 2026 Baylor Ed. 33 cross-checked Sept 2026

Chapter 10.11 — Neonatal Hematology: Erythropoiesis, Anemia/Polycythemia, Bleeding, and Neonatal Tumors

Board-review synthesis · pending expert review

Educational guideline — verify locally. Complements existing hematology/jaundice guidelines (10-01 hyperbilirubinemia, 10-02 anemia/polycythemia/thrombocytopenia, 10-09 thrombosis, 10-10 VKDB) and 13-07 Rh/ABO alloimmunization. Cross-references: jaundice (10-01), hydrops (13-07), thrombosis (10-09).

1. Clinical Overview

Clinical Overview

Neonatal haematology is developmental: nearly every abnormality is a deviation from a system switching from fetal to postnatal life. The newborn carries a high haemoglobin made largely of high-affinity fetal haemoglobin, produced by a system about to shut down temporarily; its clotting factors are physiologically low (especially the vitamin-K-dependent ones) yet balanced by low anticoagulants; and its red-cell production falters predictably after birth. Reading each disorder against that developmental backdrop — and using a few crisp discriminators (jaundice, reticulocytes, the mother's platelet count, vitamin K given or not) — resolves most neonatal haematology at the bedside.

2. Key Clinical Points

Key Clinical Points
  • Fetal haemoglobin has high oxygen affinity (left-shifted curve): loads oxygen at the placenta, unloads poorly → delivery depends on cardiac output and Hb. Beta-chain diseases (sickle, beta-thalassaemia) are silent until the haemoglobin switch; alpha-chain disorders manifest before birth (fetal anaemia/hydrops).
  • Physiological anaemia of infancy: postnatal oxygen rise switches off erythropoietin; Hb falls to a self-correcting nadir. Anaemia of prematurity is an exaggerated, earlier/deeper nadir (blunted EPO, short RBC lifespan, rapid growth) that is partly IATROGENIC — minimising phlebotomy is a real therapeutic intervention.
  • Triage anaemia by two features: jaundice separates haemolysis (present, ± hepatosplenomegaly, raised reticulocytes) from acute blood loss (absent — pale/shocked, near-normal Hb early); a LOW reticulocyte count identifies underproduction. Acute blood loss needs volume/blood (think fetomaternal haemorrhage/internal bleed).
  • Hydrops fetalis is a presentation (fluid in ≥2 compartments), mostly non-immune now; severe fetal anaemia is a leading route (immune haemolysis, alpha-thalassaemia, parvovirus B19, large fetomaternal haemorrhage). Anticipatory resuscitation (airway/effusion drainage, correct anaemia) while pursuing the cause.
  • Polycythaemia matters via hyperviscosity; causes are transfused-in (delayed cord clamping, twin-twin recipient) or produced (chronic hypoxia — IUGR, IDM, altitude). Watch for hypoglycaemia (red-cell mass consumes glucose), hyperbilirubinaemia, and NEC; partial exchange transfusion only for the SYMPTOMATIC infant.
  • Immune haemolytic disease: Rh disease requires sensitisation → spares the first pregnancy, hits later ones hard (prevented by anti-D); ABO antibodies pre-exist in group O mothers → can affect the first baby but usually milder (jaundice > anaemia). Anti-D does not cover other antigens (Kell, c, E); Kell also suppresses erythropoiesis (severe anaemia, little jaundice).
  • G6PD deficiency (X-linked; African/Mediterranean/Asian ancestry) is a leading identifiable cause of kernicterus worldwide — severe/rapid jaundice, negative Coombs; enzyme level can be falsely normal during acute haemolysis; counsel lifelong trigger avoidance. Hereditary spherocytosis: anaemia + jaundice, negative Coombs, family history, spherocytes on film.
  • Neonatal coagulation factors are physiologically low (especially vitamin-K-dependent) and tests are normally prolonged — interpret against neonatal/gestation ranges, not adult ones.
  • Two must-not-miss bleeds: (1) vitamin K deficiency bleeding — late form = catastrophic intracranial haemorrhage in a breastfed infant who missed prophylaxis; prevented by vitamin K at birth. (2) Neonatal alloimmune thrombocytopenia (NAIT) — well infant, severe thrombocytopenia, NORMAL maternal platelet count; ICH risk, can affect the first pregnancy, needs urgent matched platelets (vs maternal ITP — LOW maternal count, milder).
  • DIC is the commonest coagulopathy of the sick newborn (secondary to sepsis, asphyxia, NEC, shock): consumes platelets/factors → simultaneous clotting and bleeding; treat the underlying cause (replacement supports but does not resolve it). Newborns are the highest-risk paediatric group for thrombosis — indwelling catheters are the commonest cause; purpura fulminans signals severe protein C/S deficiency (emergency).
  • Bleeding approach: well vs sick, vitamin K given or not, platelets vs factors; remember swallowed maternal blood as a mimic (fetal vs maternal Hb testing).
  • Neonatal tumours: neuroblastoma is the commonest solid tumour of infancy (abdominal mass; certain newborn forms regress spontaneously); sacrococcygeal teratoma can cause fetal high-output failure/hydrops; congenital leukaemia is rare and associated with trisomy 21 (transient abnormal myelopoiesis often self-resolves but needs follow-up; lifelong increased leukaemia risk). Neutropenia in a septic newborn reflects severity (exhausted reserve), not safety.

2+. Sacrococcygeal Teratoma (Baylor Ed. 33)

Recognition, risk and management
  • The commonest neonatal tumour — about 1 in 35,000–40,000 live births, female:male 3:1. Most are diagnosed antenatally by ultrasound; fetal MRI separates SCT from myelomeningocele and other sacral lesions.
  • Altman classification: type 1 mainly external; type 2 external with an intrapelvic part; type 3 mainly intrapelvic and intra-abdominal with a small external part; type 4 presacral with no external component.
  • Fetal risk comes from size and vascularity: poor outcomes are linked to high vascularity, solid tumour and a tumour volume to fetal weight ratio above 1.2. Large vascular tumours cause polyhydramnios with preterm birth and high-output failure with placentomegaly or hydrops — follow with serial ultrasound. Hydrops or preterm labour may prompt fetal intervention (open excision or debulking, endoscopic laser ablation).
  • After birth: NICU care may be needed for prematurity, high-output failure, DIC, or tumour rupture or bleeding. Haemorrhage from a large SCT is uncommon but highly lethal — a temporary tourniquet around the tumour base can be lifesaving on the way to theatre. Place IV access in the upper limbs (the lower limbs will be in the surgical field; see chapter 14.7). Resect once stable; prognosis depends on malignancy and complete resection.
  • Follow-up: most recurrences occur within 3 years — examine and check AFP and CA-125 every 3 months for at least 3 years.

3. References to Verify

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