Iron deficiency is the most common cause of anaemia in childhood. Potential sources of blood loss should be excluded (eg recurrent epistaxis, inflammatory bowel disease or Meckel's diverticulum). Otherwise the most common reason is dietary.
Iron deficiency is associated with developmental delay, so there is an argument for treating even when the haemoglobin itself is not particularly low. But an isolated low ferritin is not very meaningful, if you use a gold standard for deficiency of a large response to iron supplementation! Low total or mean cell haemoglobin in isolation is a specific marker of iron deficiency, but other markers are only predictive when found abnormal values seen in 2 or more of MCV, ZPP and/or ferritin. These all, with the exception of ferritin, correlate moderately with each other. Charlotte Wright, Arch Dis Child 89(11):1028-31, 2004 PMID 1549905
Normal newborn has 90%+ HbF (alpha-gamma chains), dropping with age. In childhood, normal HbF is <2%. HbA2 (alpha-delta instead of alpha-beta) is normally <3.5%, and HbA:HbA2 ratio should be at least 40:1.
4 genes for alpha chains. Deletions are extremely common in the Mediterranean, Africa, SE Asia but actually Africans tend to carry the "α-" defect not the "--" defect that predisposes to disease.
Electrophoresis will show high quantities of Hb Barts in thal disease. In the trait form, there are often normal levels of HbA so need to do gene deletions.
Seen in the Mediterranean, Africa, SE Asia. Just 2 genes, so a heterozygote is a carrier, may have mild microcytic anaemia. In the Mediterranean form, the gene is usually a zero producer, so the disease is more severe than in Africa, where production is reduced but not absent.
Beta thal Major (homozygous) present after 6 months of age with anaemia, frontal bossing, growth failure, hepatosplenomegaly.
In Beta-thal trait, HbA2 is obviously increased eg 3.5-7%, and ratio reduced eg 20:1. The only exception is if there is co-existing severe iron deficiency. In beta-thal disease, you may not see such an increase if there's lots of HbF around (about half the cases), and increased HbF is not specific; but the clinical picture doesn't leave much doubt. Inclusions (alpha chains) seen esp post splenectomy. Retic count surprisingly normal due to severe intramedullary erythroblast destruction.
Treatment is with folate and regular blood transfusion, which then leads to cirrhosis, pigmentation, diabetes etc. Splenectomy reduces transfusion requirements but because of the increased vulnerability to pneumococcal infection this is usually deferred until later childhood.
Deferipone is oral chelating agent, used in Europe but not licensed in US. Acrimonious debate between lead researcher and drug company about research.
An intermediate beta thal exists, where transfusion is only required at times of increased stress.
Combined alpha thalassaemia and sickle cell disease tends to give less anaemia, but more vaso-occlusive crises.
Lower limit of normal is 8 in the first 48hrs (!), 1 in infancy, 1.5 in childhood, 1.8 in adulthood.
Clinically, periodontitis and aphthous ulcers are seen at 0.5 and below, poor wound healing may be the presenting sign. Superficial candidiasis is often seen but probably associated with frequent antibiotic use rather than a defect!
In Cyclical neutropenia, the count drops below 0.2 every 3 weeks or so. = Elastase defect (heterozygous). Various genes identified incl WASP (same gene as Wiskott-Aldrich, where low platelets also seen). Alternatively, a block in the promyelocyte stage will block neutrophil maturation. There may be compensatory monocytosis or eosinophilia.
Described initially in Northern Sweden, also common in Kurdistan. Severe autosomal recessive congenital neutropenia, presents in neonatal period with fever, local infection esp omphalitis, skin and abscesses. G-CSF is protective, although levels and binding are normal; acts by preventing excessive apoptosis of progenitors. When first introduced, neutrophil count may start to cycle! Needs big doses cf other indications eg 5-20, up to 60-100mcg/kg, and even then periodontitis can be problematic. Myelodysplastic syndrome and myeloid leukaemia are associated, esp in the presence of monosomy 7.
Adolescents with ALL do better on paed protocols! National cell bank just starting. DXM better than pred. 6MP less SE's than thioguanine.
ALL 2003 - Minimal residual disease is clear morphologically at the end of induction, cf DNA techniques. Indicates speed of response, which is prognostic. Most relapses come from low risk children! Hence need for other predictors of potential relapses. MRD pos have 50pc relapse rate so get delayed intensification. MRD neg have 5pc rate, so decrease treatment (if parents consent! Difficult!). Most benefit seen in young girls with lgw counts. End result is 3x as many schedule C (most intensive), but cf cost of BMT.
Tumour lysis syndrome:
Hyperviscosity:
Spinal cord compression - pain, weakness, lower back tenderness but not necessarily all. Rx Dexamethasone.
Encephalopathy from methotrexate, tacrolimus.
Acute GVHD ie within 3/12:
Platelet problems:
British Journal of Haematology, 2003, 120, 574596
80% of children with ITP will recover spontaneously within 68 weeks.
Diagnosis by exclusion. Can occur at any age, but in neonates maternal ITP or alloimmune thrombocytopenia more likely.
In acute ITP:
May follow an acute viral infection or within 6 weeks of immunisation esp MMR (1 in 24 000 risk).
(The CSM recommend that children who develop MMR associated ITP should have serology checked and a second dose given if not fully immune rubella associated ITP is a bigger problem than MMR associated ITP!).
ITP associated with Varicella needs special caution: occasionally more complex coagulation disorders viz antibodies against proteins S +/or C.
A chronic history, with symptoms developing over weeks or months, is possible in ITP but suggests something else. Beware non-accidental injury (NAI) and meningococcal disease - children with infection usually have other features and non-accidental injury does not present with generalized purpura.
Classify clinically and not by platelet count, because even with severe thrombocytopenia (less than 10 /fl) clinical symptoms usually "mild". Equally, pronounced skin purpura and bruising, however extensive, do not indicate a serious bleeding risk on their own and serious complications are probably rarer than sometimes quoted.
Several therapies raise the count faster than no treatment. However, all have significant side effects and none alters the underlying pathology nor increases the chance of complete remission. These strategies are appropriate for children with severe bleeding symptoms.
Recommendation: If a child has mucous membrane bleeding and more extensive cutaneous symptoms, high dose prednisolone 4 mg/kg/d is effective (Grade A recommendation, Level Ib evidence). It can be given as a very short course (maximum 4 d). There are no direct comparisons of low dose (12 mg/kg/d) with high dose therapy. If lower doses of 12 mg/kg/d are used the treatment should be given for no longer than 14 days, irrespective of response.
Other steroid regimens:
Intravenous immunoglobulin is effective and seems to work more quickly cf steroids (mean 2 days to achieve plt count of 50 cf 4 days). Works by binding to spleen receptors, reducing platelet destruction. Expensive and invasive, reserve for emergency treatment of patients who do not remit or respond to steroids and who have active bleeding. It is an appropriate treatment to enable essential surgery or dental extractions. IVIg is a pooled blood product, the risks of which must be explained to patients. It has significant side effects, (75% of children, esp severe headache).
Recommendation: IVIg can raise the platelet count rapidly, but should be reserved for emergency treatment of serious bleeding symptoms or in children undergoing procedures likely to induce blood loss. It is effective given as a single dose of 0.8 g /kg (Evidence level Ib, Grade A recommendation). Lower doses are also effective, and fewer side effects are seen, in younger children; but usually it is used for emergencies where a higher dose eg 1g/kg may be more appropriate.
Anti-D immunoglobulin is less expensive than IVIg and can be given to Rh (D) positive individuals as a short infusion, and is therefore amenable to outpatient therapy. It is as effective as IVIg in children when given at sufficient dosage (4550 lg /kg), but the mechanism of action is not fully understood. Like IVIg, anti-D is a pooled blood product; some degree of haemolysis is commonly seen, occasionally severe and is associated with renal failure. Lower dose treatment is less effective at raising the platelet count than IVIg.
Life-threatening haemorrhage is the only indication for platelet transfusion in ITP, a destructive platelet disorder where transfusions of normal doses are unlikely to be effective. In a life-threatening situation (such as the rare ICH) larger than normal doses are required with monitoring of the increment as a guide, and other modalities such as high dose IV steroids and IVIg should be given at the same time to maximise the chances of raising the count and stopping the haemorrhage.
The management of children with continuing thrombocytopenia is essentially the same as for acute ITP. Many children settle with an adequate platelet count (i.e. more than 20 /fl) and have no symptoms unless injured. In children under 10 years of age at diagnosis spontaneous remission is likely to occur eventually eg within 15 years; expectant management can continue.
Children more than 10 years of age at diagnosis, esp girls, are more likely to sustain a chronic course but tends to attenuate over time.
Most children need no specific therapy to raise the count unless injured or requiring surgery or dental extraction. Particular problems may arise for girls at the onset of menstruation. It is advisable for the family to carry a card, letter or medical bracelet with details of the disorder in case of emergency eg trauma.
Children with counts persistently below 10 /fl are likely to have some symptoms, e.g. easy bruising or odd petechiae. Very rare, and are difficult to manage - refer such chronic severe ITP (CSITP) cases to paediatric haematologists with a special interest.
A significant group of children with ITP have counts of 1030 /fl, and although they have no serious bleeding, are nevertheless troubled by purpura esp physical appearance, secondary school. Lifestyle issues and restrictions on sporting activities become more important and should be taken into account in considering therapy. Intermittent treatment with IVIg can be given to cover activity holidays after appropriate discussion of the risks.
Splenectomy is often considered, but it is ineffective in around 25% of cases, and most chronic cases remit spontaneously. It does bump the platelet count up with fewer symptoms but it is clear that the relapse rate with longer follow-up is high. Given that the risk of dying from ITP in childhood is extremely low (less than 1 in 500), that the mortality associated with splenectomy is 1.4 to 2.7% and that the risk of over-whelming sepsis probably persists for life, splenectomy is only justified in exceptional circumstances eg life-threatening bleeding.
Intravascular (eg DIC) or Extravascular (eg hypersplenism)?
Immune or non-immune? Do Indirect Coombs test.
Paroxysmal cold haemoglobinuria - most common autoimmune haemolytic anaemia in under 3 yrs. Post viral. NB normal platelets no fragments on film cf HUS. Donath-Lansteiner antibody. ?assoc with renal failure. Plasma exchange helps.
Phenotype (severity) consistent within family, but very different between families. FH may be vague eg splenectomy, jaundice rather than awareness of underlying diagnosis... Anaemia, jaundice and splenomegaly classically. Splenomegaly is usually mild and there is no increased risk of rupture. Neonatal jaundice can be severe but does not predict severe disease! Severe cases (assessed when well, not during crises, only about 5%) can be transfusion dependent in first years of life (erythropoietin helpful) but not usually afterwards. May present with parvovirus aplastic crisis (not just red cells; white cells and platelets often drop too) only happens once.
Diagnosis by spherocytes on film, reduced MCV, high reticulocytes (but retics do not go up during aplastic crisis), unconj hyperbilirubinaemia, splenomegaly. There are other causes of spherocytes, and they can be seen in normal neonatal blood films. Differential is autoimmune haemolytic anaemia, which is associated with acute viral infection (direct Coombs test usually positive). Osmotic fragility test does not distinguish, can be false negative in iron deficiency, and is unreliable in the first few months of life. New EMA binding test takes 2 hours and is 92% sensitive. Gene tests don't add much.
Other problem is gallstones. High reticulocyte count predicts.
Folate probably only necessary for severe cases.
Most children are asymptomatic, but severe cases can have growth failure, lethargy, heart failure and leg ulcers. Should be delayed until at least 5 yrs, potentially laparascopic and/or partial. Do cholecystectomy at same time if symptomatic. Platelets rise to abnormally high levels after splenectomy, but no apparent increase in thrombosis.
Arch Dis Child PMID 15321852
Autosomal recessive genetic disorder caused by a single nucleotide mutation of the haemoglobin ί-unit. The 6th amino acid of the resulting polypeptide becomes the hydrophobic amino acid valine instead of hydrophilic glutamic acid. The mutant protein giving rise to a defective variant of haemoglobin, called haemoglobin S (HbS). The variant is suceptible to polymerisation in cooler or more hypoxic conditions, the precipitated haemoglobin distorts causing it to resemble a sickle and ultimately damages the erythrocyte causing haemolysis.
The gene is more common among Africans and is occasionally seen in races from the Middle East and South Asia. Its prevalence is due to the relative resistance to malaria seen in sickle cell trait (the heterozygote form), due to shortened red cell lifespan. It may of course co-exist with other genetic disorders of haemoglobin structure.
Sickling is triggered by various factors, including a cold environment, acidosis, hypoxia and hyperviscosity eg dehydration. Blood flow in capillaries is impaired, which leads to a vicious cycle of increased tissue acidosis and hypoxia. Infarction may occur if the impairment of blood supply is sufficiently severe and acute.
Can be diagnosed even in newborns by hemoglobin electrophoresis, liquid chromatography or DNA analysis. These tests are pretty equivalent, so whatever is used locally. There are solubility testing methods (Sickledex, Sicklequik) but these are not appropriate for diagnosis - although they identify sickle haemoglobin, they miss haemoglobin C and other genetic variants. Furthermore, solubility testing is inaccurate in the newborn, in whom fetal hemoglobin is overwhelmingly predominant, and false negatives seen in severe anemia.
Screening programmes exist in many US states RCT showed 84 percent reduction in the incidence of pneumococcal sepsis, when prophylactic oral penicillin was initiated by the age of three months.
Presents in infancy or early childhood. The neonate with its high proportion of Haemoglobin F does not get symptoms until there is enough abnormal haemoglobin A produced for red cells to start to sickle.
Pain can be widespread, but particularly involves bones, the spine, the chest.
Chest involvement may mimic pneumonia, with unilateral or bilateral signs of consolidation, pleuritic pain, and hypoxia. Pain, usually in chest wall, thoracic spine and upper abdomen leads to hypoventilation, with atelectasis and subsequently worse sickling. ACS has a high mortality, so treatment should be prompt on suspicion of the condition. Hypoxia should be managed aggressively, with respiratory support if necessary.
Usually secondary to Erythrovirus B19 (formerly known as parvovirus B19) infection, which can trigger transient bone marrow arrest and hence exacerbation of chronic anaemia with an absence of reticulocytes. Classic "slapped cheek" appearance may never become apparent. Can affect multiple members of a family simultaneously. Differential is spleen sequestration.
Manifest as anorexia, abdominal pain, distension. Usually not diarrhoea or vomiting. Usually not rebound. Bowel sounds usually quiet.
Typically affects middle cerebral artery territory but may affect any region of the brain; may be transient or permanent. Seizures may occur. Predictive factors are:
Differential is meningitis, subarachnoid haemorrhage (associated with multiple intracranial aneurysms).
Although anaemia is common in SCD, repeated transfusions lead to the possible complications of:
Hence top up transfusion is only used for acute symptomatic anaemia eg cardiac failure, severe sequestration or pre-operatively. Do not transfuse above Hb 11g/dl. Regular transfusions have a prophylactic role - see on-going treatment below.
Hyperhaemolysis is a life threatening complication of red cell transfusion in sickle cell disease. Can be acute (within 7 days of transfusion) or delayed. Affects not just transfused but autologous cells so Hb can drop below previous level. Fever, haemogobinuria as usual; negative DAT, reticulocytopenia seen (cf parvo). Cover subsequent transfusion with IVIG and steroids; use erythropoietin to maintain. For elective surgery, prophylactic postop CPAP has been used without transfusion. Risk factors poorly defined.
Undertaken to rapidly reduce the percentage of sickle cells in the circulation where life-threatening eg severe chest syndrome, stroke, multi-organ failure. The aim is to reduce %HbS to <20%. Complications are common eg fluid overload, transfusion reaction.
Causing the usual problems of cholecystitis, biliary colic, jaundice etc. Stones are radio-opaque in up to 50% of cases. Some patients develop jaundice without gallstones, and this is thought to be due to intrahepatic sickling.
Typically of hips and shoulders, although symptoms may involve groin and knee. Causes chronic pain and ultimately joint damage with restriction of movement. Treatment is difficult. Transfusion pre/post-operatively is mainly to prevent progression in contralateral joint!
Consider for:
Options:
Iron overload can be monitored by means of Ferritin levels. Chelation therapy should commence at ferritin levels of 1000mcg/l, with desferrioxamine (desferal) the chelator of choice. Treatment should include vitamin C. Ophthalmological, audiological and cardiological review is necessary.
A group of disorders, characterized by :
The common factor is the inability to kill infected target cells. The resulting sustained natural-killer-cell and cytotoxic T-cell activity leads via inflammatory cytokines to macrophage activation, dissemination, and organ infiltration. Hemophagocytosis in the marrow is not always evident at presentation so serial bone marrow examinations may be required.
A spleen aspirate may be an alternative to marrow.
There are four categories:
NEJM 2004:351
Treatment - HLH94 regimen then ciclosporin for control, BMT for cure. Henter diagnostic criteria.
Immune mediated (IgG vs heparin - platelet factor complex), approx 1 in 100 patients receiving unfractionated heparin for more than 5 days. Platelet activation occurs, with thrombosis and consumption. Endothelial injury may also occur. Most commonly seen in cardiovascular surgical patients getting unfractionated heparin, rare in pregnancy!
Differential non-immune heparin induced thrombocytopenia (mild, within 4 days of starting); mechanical; other drugs; antiphospholipid; DVT; SBE.
Assay ELISA is sensitive but not very specific. Treat by stopping all heparin and starting alternative anticoagulation (but not warfarin, can worsen!) Argatroban, Lepirudin, Danaparoid (although risk of cross reactivity, as a heparin derivative). Kids may need bigger doses than adults. Glycoprotein IIb/IIIa inhibitors reduce platelet aggregation but not activation. Avoid platelet transfusions.
Circulation Volume 111(20), 24 May 2005, pp 2671-2683
Basic thrombophilia screen is:
The basic coagulation screen can show whether there is abnormal clotting - a prolonged APTT which only partially corrects on mixing with normal plasma suggests an anticoagulant. The more advanced coagulation tests are:
An autoimmune disorder of recurrent thrombosis and pregnancy loss! Not well defined in children, usually heralds SLE and is probably much the same thing. A mixed bag of conditions, despite the name, where there may be antiphospholipid antibodies (of one sort or another) including anticardiolipin and lupus anticoagulant (persistently).
Diagnosis is usually made by initially demonstrating a prolonged APTT that does not correct on mix (ie anticoagulant), which is then shown to be anti-phospholipid in nature (abnormal DRVVT, or anticardiolipin). Various targets are involved eg Anti B2GP-I ([beta]2glycoprotein 1) antibodies, may be useful in the diagnosis if traditional assays for anticardiolipin/Lupus anticoagulant are negative.
Lupus anticoagulant is the name for a phospholipid related anticoagulant (not cardiolipin) found on testing. A total misnomer, as it is PRO-thrombotic and only occasionally associated with lupus.
If found incidentally, then likely to be transient. Even if persistent, thrombosis is unusual. If no suggestion of lupus, prophylaxis is probably unnecessary, otherwise low dose aspirin seems reasonable. Thrombosis can be arterial OR venous. Other associated features are:
Clearly overlaps with lupus. There are various other associated features, generally not associated with thrombosis! But thrombosis can be catastrophic viz microangiopathy, pulmonary haemorrhage, ARDS, cerebral/adrenal infarction, Budd-Chiari (hepatic vein).
Acute treatment is with IV or subcut heparin, followed by warfarin (target INR 2-3). Aspirin may be added if problematic. Hydroxychloroquine should probably always be used. Avoid smoking, oestrogens, cocaine... Plasma exchange and Rituximab (?) may be used in life-threatening cases.
The antibodies act by inducing adhesion molecules from endothelial cells, upregulating tissue factor, activating platelets and the complement cascade generally. Current Opinion in Hematology. 13(5):366-75, 2006
The clinical severity of a patient's hemophilia is gauged by the baseline clotting factor level, a value that remains fairly constant throughout that person's life, and is pretty consistent through a family. Around 5% of normal is moderate.
Intramuscular injections should be avoided if at all possible. If they must be given, factor replacement therapy should precede the injection. Parenteral agents should be given intravenously or subcutaneously. Immunizations are administered subcutaneously.
Aspirin is contraindicated due to antiplatelet effect. Avoid NSAIDs if possible, and contraindicated if actively bleeding or being treated for a recent bleeding problem.
The treatment of choice for individuals with factor VIII deficiency is recombinant factor VIII. There are different brands, with different combinations of additives which may or may not be antigenic. Cryoprecipate and fresh frozen plasma are used only if factor cannot be obtained.
For individuals with Christmas disease (factor IX deficiency), recombinant factor IX is the treatment of choice. Fresh frozen plasma is only used if factor is unavailable.
Ideally let patients/parents reconstitute and administer their own factor. Families should have an emergency dose of factor concentrate or DDAVP in their home and to take it with them when they travel, esp if they do not live close to a hospital. Factor replacement should be offered within 1 hour of the patient's arrival: it should be readily available, the patient should not be left waiting, and treatment should not be deferred pending x-rays or other results. The most experienced IV therapist or phlebotomist available should perform venipuncture - misses and tourniquets may cause painful hematomas which then limit further IV access - use the smallest needle possible unless volume replacement is needed..
Factor replacement must be administered intravenously by IV push over 1-2 minutes. Round the dose up to the closest full vial ie don't throw away any left overs (excess factor does not create a hypercoaguable state but will prolong the therapeutic half-life of the product administered).
For individuals with inhibitors (antibodies to factor VIII or IX), treatment decisions may be more complicated. The care of inhibitor patients should be urgently discussed with the patient's hematologist. If an individual with an inhibitor presents in a life- or limb-threatening scenario, the safest immediate action is to prescribe recombinant factor VIIa (rFVIIa, Novoseven) at a dose of 90 mcg/kg or activated prothrombin complex concentrates (FEIBA, Autoplex) at 75-100 units/kg (contraindicated in factor IX patients with a history of inhibitors and anaphylaxis). Hopefully the patient/family can provide information on response to second line therapeutic agents.
Medical and Scientific Advisory Council (MASAC) of the US National Hemophilia Foundation 2003
Presents with mucous membrane bleeding (eg epistaxis, ecchymoses, menorrhagia, and excessive bleeding with surgical or other invasive procedures esp post adenotonsillectomy). Not usually petechiae (more commonly associated with platelet function defects) but may be seen esp if aspirin/NSAID has been taken. Not usually haematomas (more characteristic of hemophilia) but may be seen if severe vWD. Menorrhagia is a frequent presentation in women.
vWD has been reported in association with hemorrhagic telangiectasia (Osler-Weber-Rendu) syndrome; so consider telangiectasias contributing eg recurrent epistaxis or gastrointestinal bleeding.
Diagnosis may be missed esp in mild vWD, because vWF levels are influenced by factors such as age, inflammation, stress, pregnancy, hormonal cycles, hypo- or hyperthyroidism, and various medications. Hence stress and inflammation due to illness (eg ruptured appendix) may reduce symptoms, whereas minor surgery in the nonstressed patient may be associated with severe bleeding! Family history should be positive, as VWD is inherited in autosomal dominant fashion.
Although men and women are affected equally, women may be recognized more often because of menorrhagia and excessive bleeding with childbirth.
vWF binds platelets in plug formation. It is also a carrier for Factor VIII - without it factor VIII is lost from the circulation. Subtypes:
Bleeding time is not particularly sensitive or specific. Prothrombin time is normal. APTT may be prolonged, depending on the impact on factor VIII. Ristocetin stimulates platelet aggregation in presence of vWF so is a more sensitive and specific test.
Desmopressin (DDAVP) is available in IV form and concentrated intranasal form (not the same as used for enuresis and diabetes insipidus!).
Several vWF concentrates are available, with different ratios of vWF to Factor VIII - used to cover surgery. Maximal increase in VWF/FVIII occurs at 30 to 60 minutes, so time the infusion as close as possible to the surgical procedure. The response time varies between individuals and tachyphylaxis (ie reduction in effect) may occur with repeated doses of desmopressin. A trial to establish effectiveness (judged by vWF:RCo (Ristocetin) level) may be helpful.
For major surgery, give concentrate every 12 to 24 hours for 2 to 3 days to achieve levels of VWF greater than 40%. In patients who have decreased baseline VWF:RCo, levels should be measured daily and extra doses concentrate should be administered if tachyphylaxis occurs. In patients in whom FVIII:C levels also are decreased, FVIII:C should be monitored and maintained at a hemostatic level as recommended for hemophilia A. In Type 2B give platelets if low count.
Antifibrinolytics are useful esp for oral/nasal mucosal bleeding. Can even be used topically (mouthwash).
In children generally associated with sepsis. Syndrome of deranged clotting, low platelets, haemolysis. Blood film may be informative. Treatment is of underlying cause and support with blood products. Recombinant activated factor VII has been used successfully in pulmonary haemorrhage associated with sepsis related DIC.
All blood products are now leucocyte depleted. This effectively makes them CMV safe, but since individual bags are not tested, in high risk situations CMV negative blood may still be appropriate eg marrow transplants and cellular immunodeficiency. All pooled products are from non-UK donors. There is still uncertainty about the safety of large volume SAGM blood transfusions in neonates and management of T activation.
All products survive 4.5 hrs out of fridge. Irradiated blood appropriate for prior to stem cell transplant, Digeorge & other T-cell immunodeficiencies (or suspected eg lymphopenia). Young blood can be prescribed for high k+ recipients eg renal. Neonates under 4/12 don't need repeat grouping after transfusions! Platelets need oxygen so remove from any packaging, but keep agitated and Tx over 30 mins. Give Cryo/FFP over 1 hr. Cryo 5-10ml/kg! cf FFP I0-20ml/kg. Group compatible takes 10-30 mins to crossmatch. 0bservations should be done every 15 min intervals after starting each unit, most reactions occur within 15 min.
Each unit blood costs £150. Check name bands! No prelabelling of samples.
Early check @15mins will detect most major reactions! Anxiety, hypothermia, pain @ infusion site, haematuria, loin/back pain, resp distress are signs of major reaction but fever/rigors/rash/urticaria, hypertension can precede major reaction. Delayed reaction: fever, jaundice, poor increment, dark urine.
Commonest non-brain tumour in childhood. Arises in cells of neural crest ie sympathetic chain - which means they can be just about anywhere. Mostly affects young children.
Specific presentations:
Rarely goes to lungs!
Diagnosis by testing urine for catecholamines, esp HVA and VMA (RHSC lab looks for phenols). MIBG isotope scan may be useful for finding secondaries (common at presentation). Stage 2 is single tumour with incomplete resection (2B has positive lymph node). Stage 3 is single tumour crossing midline or contralateral lymph node positive. Prognosis is not just dependent on stage however - age, microscopic appearance, and MYCN oncogene status contribute to very different outcomes. Some stage 4 neuroblastoma with widespread tumours in very young infants will resolve spontaneously.
This work
is licensed under a Creative
Commons Attribution-Noncommercial-Share Alike 2.5 UK: Scotland License.