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Haematology
Dr Dániel Erdélyi2nd Dept PediatricsSemmelweis University
Haematology topics for final exam
• Coagulopathies• Leukaemia• Hodgkin’s disease, Non-Hodgkin’s lymphoma• Diseases with hepatomegaly, splenomegaly• Lymphnodes enlargement - DD and
treatment• Anaemias• Iron deficiency anaemia
1st case presentation
6 mo. old boy C/O ↑crying, progressively over recent days.O/E well developed and hydrated, CVS stable,Abdo distended, liver 1.5–2 cms below costal margin,Exam unremarkable otherwiseWBC 14.3 G/L, Neutro 13%, Lymph 76%Hb 102 g/L, Plt 379 G/LCreat 25 umol/L, U&Es-CRP normalIdeas, please!
Lab values normal for adults would be very worrying in a baby!
1. LAB NORMAL RANGES
Normal Hb in infancy & childhoodHb (g/L)
Age
100
150
200
boysgirls
3 mo 6 mo 10 y
Hb (g/L)
Age
100
200
3 mo 6 mo
Age Hemoglobin (g/l)
3 mo - 2 years < 100
< 110
6 - 14 years < 120
Adult women < 120
Adult men < 130
2 - 5 years
Anaemia, thresholds
Normal WBC in infancy & childhood
15
WBC (G/L)
Age
10
30
3 mo 6 mo
5
10 y
Differential count in children1 wk
4 y
10 y
Neutrophils Lymphocytes
Normal platelets in childhoodPlt (G/L)
Age
150
300
„Adult type” FBC in 6 mo baby(case presentation – to interpret)
• Hb 140 g/l• WBC 4.5 G/L• Neutrophils 70%
2nd case presentation
18 mo. girl Pale, GP requested FBCWBC 12.5 G/L, Neutro 26%, Hb 82 g/L, MCV 59 fl, RDW 18%, Plt 530 G/L,Physical exam unremarkableInterpretation, DD, please!
Classification of anaemia
1. Blood loss
•acute•chronic – iron deficiency later
•external•internal
2A. Haemolytic - intrinsic•membrane-defects (sphaerocytosis, elliptocytosis, stomatocytosis, PNH)•hemoglobinopathy (sickle cell anaemia, thalassaemia, rare others)•enzyme-defects (pyruvate kinase defect, glucose-6-phosphate dehydrogenase def., other rare forms)
2B. Haemolytic – extrinsicImmune Autoimmune haemolytic anaemiaAlloimmune (neonatal)Autoimmune diseasesTransfusion reactionsNon-imm. Infections (malaria, mycoplasma, EBV)
+/- immune mechanismsHypersplenismMicroangiopathic (HUS, TTP, DIC,
Kassabach-Merritt syndrome)
Not typically called as haemolytic anaemia
3. Hyporegenerative•Haematinics: Fe, Cu, folate, B6, B12, protein...•Primary or idiopathic: congenital anaemias, SAA•Temporary, by external causes:
viral infections (Parvo B19, EBV, CMV, else) drugs (ibuprofen, metamizol; chemotherapy)
•Bone marrow space ↓leukaemia, neuroblastoma, osteopetrosis...
•Secondary other: chronic infections,renal, hepatic, endocrine disordersautoimmune (SLE)
2. DIAGNOSTICS
Microcytic Normocytic Macrocytic
Iron deficiency Blood loss B12 folate deficiency
Chronic disease Haemolysis/AIHA Liver diseaseThalassaemia Bone marrow Aplastic
failure MDSLead intox. Renal failure Congen dyserythr. aB6 deficiency Hypothyroidosis
Gravidity
Various: corpuscular haemolytic anaemias
RDW: red cell distribution width
• Normal: 11-14%• Increased: iron deficiency, cong. membrane
disorders, burns, microangiopathic
Hypochrome / Hyperchrome
• Usually correlate with micro/normo/macrocytic featire
• Sphaerocytosis: ↓ MCV ↑ MCHC
Reticulocyte count
• Normal: 0.5 – 1.5 %, but consider anaemia• High: bleed, haemolysis• Low: hyporegenerative
Other lab tests to identify haemolysis:• Billirubin• LDH, haptoglobin• Consider intra/extravascular haemolysis
Blood film
Iron deficiency
Thalassaemia
Sphaerocytosis
Elliptocytosis
Megaloblastic anaemia
Fragmentocytes
Sickle cell disease
Lead intoxication
Bone marrow cytology/histopath.• For hyporegenerative anaemias only
Antibodies• For haemolytic anaemias only• Coombs: direct (DAT) / indirect antibodies • Cold and warm antibodies• Specific antibodies
Miscellaneous others
• Haemoglobin electrophoresis / HPLC• Osmotic fragility / cryoresistance• ADAMTS13 activity• D-dimer, coagulation factors• Haemoglobinuria• Erythropoietin
3. CLINICAL COURSE @ THERAPY
• Prelatent / latent deficiency / anaemia• Causes• Symptoms: haematology, others• Lab signs; DD chronic disease related a.• Therapy– iron salts (ferrous sulphate II, III)– iron carbohydrate complex– iv iron
• Follow up
Iron deficiency anaemia
Haemolytic anaemias
Clinical signs:•Pallor, collapse•Icterus•Splenomegaly•Gall stones•Possibly emergencies:
rapid haemolysis, aplastic crisis
Immune-haemolytic anaemia
• Course of AIHA• Therapy:– glucocorticoids– IVIG– other immunosuppressive therapies, rituximab– splenectomy– transfusion only when life-threatening
www.wikipedia.org
Haemoglobinopathies
Sickle cell anaemia – special points
• Painful cryses• Sequestration• Hydration• Analgaesia• Hyposplenism• Transfusion policy• Exchange transfusion• Transcranial Doppler-US• Hydroxyurea, allo-HSCT
Congenital aplastic/hypoplastic anaemias
• Fanconi anemia• Dyskeratosis congenita• Shwachmann-Diamond sy• Constitucional aplastic anemia• Diamond-Blackfan anaemia• ...
• DNA repair defect• Mean age at diagnosis 9
years, pancytopenia• Multiple anomalies
possible: short stature, bone, kidney, heart, malformations, skin pigmentation, hypogonadism, learning difficulties
Fanconi anaemia
• Ribosomal defect• Isolated red cell aplasia, presents at few
months of age• Most children without other
abnormalities
Diamond-Blackfan anaemia
Treatment of congenital aplastic / hypoplastic anaemias
• Symptomatic: transfusions, kelating agents • Steroids• Allogenic stem cell transplant
4. LEARNING POINTS
Frequent, important...
Anaemia - most important:•Iron deficiency: dietary, cow-milk protein allergy, malabsorption•Anaemia in chronic diseases
2nd line•B12 and folate deficiency•Corpuscular (e.g. sphaerocytosis) and autoimmune haemolytic anaemias•In Hungary, haemoglobinopathies are rare
Bleeding - most important:•ITP, DD of thrombocytopenia•Haemophylia A, B•von Willebrand’s
2nd line•DIC, HUS•Lupus anticoagulans and other inhibitors
Haemat. malignancies – most important:•ALL >> AML >> CML •Hodgkin’s•Non-Hodgkin:–Burkitt’s– Lymphoblastic lymphoma
Specific to / different in children
Alloimmune cytopenias in newborns+ thrombocytopenia from maternal ITP
Vitamin K deficiency in infantsPresenting symptoms of congen. coagulopathiesFrequency of malignanciesApproach to polycytaemia, thrombocytosis
5. ELSE
• New leukaemic patient• New malignancy• Febrile neutropenia