Anemia. Classification and Diagnosis
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Anemia. Classification and diagnosis B. Rosich del Cacho*, Y. Mozo del Castillo** *Consultant physician, Pediatrics Service, Hospital Universitario Joan XXIII, Tarragona. **Consultant physician, Pediatric Hemato-Oncology and Hematopoietic Stem Cell Transplantation Service, Hospital Universitario La Paz, Madrid Abstract Resumen Anemia is defined as a reduction in hemoglobin La anemia se define como una reducción de la concentration below normal levels for age, gender concentración de la hemoglobina por debajo de los and ethnicity. It is the result of an imbalance niveles considerados normales para la edad, sexo y raza. between the production and destruction of red Es el resultado de un desequilibrio entre la producción y blood cells, which characterizes or accompanies la destrucción de hematíes, que caracteriza o acompaña a various conditions. It is the most common diferentes patologías. Se trata del problema hematológico hematological abnormality in childhood, the más frecuente en la infancia, cuya causa principal es la main cause of which is iron deficiency. Clinical ferropenia. Con frecuencia, las manifestaciones clínicas manifestations are often nonspecific. Diagnosis son inespecíficas. El diagnóstico comienza con: un begins with a full blood count, peripheral blood hemograma, el frotis de sangre periférica y los parámetros smear and biochemical parameters of hemolysis bioquímicos de hemólisis y del metabolismo del hierro. and iron metabolism. The overall diagnostic Se revisa la aproximación diagnóstica general del niño approach of the child with anemia is here con anemia, proponiendo un enfoque basado en un reviewed, and an algorithm is proposed based on algoritmo a partir de los datos hematológicos básicos. basic hematological data. Finally, a brief list of Finalmente, se aporta un breve listado de referencias references is provided. bibliográficas básicas. Key words: Anemia; Child; Infant; Classification; Diagnosis. Palabras clave: Anemia; Niño; Lactante; Clasificación; Diagnóstico. Introduction or hematocrit in peripheral blood below the following Hb thresholds according Anemia is defined as a reduction in the 2 standard deviations (-2 SD) for the to age group: concentration of hemoglobin or hematocrit, age, sex and ethnicity of the patient • 11 g/dl in children 0.5-4.99 years-old the normal levels of which depend on age, (Table I). (preschool age). sex, and ethnicity. Iron deficiency anemia • Hemoglobin (Hb): complex protein • 11.5 g/dl between 5-11.99 years of age. is the most prevalent hematologic disorder made up of heme groups containing • 12 g/dl between ages 12-14.99 years. of childhood. iron and a protein portion, globin. The concentration of this erythro- The global prevalence of anemia n this article, the general concepts cyte pigment is presented in grams was 47.4% ([95% CI] 45.7-49.1) in of anemia in childhood and its diag- (g) per 100 ml (dl) of whole blood. preschool-age children and 25.4% ([95% I nostic approach are reviewed. Iron • Hematocrit (Hct): fraction of the CI] 19.9-30.9) in school-age children. deficiency anemia (the most common volume of erythrocyte mass with The prevalence in preschool children cause of anemia in the pediatric age) respect to total blood volume. It is varied widely by country, led by coun- and hemolytic anemia are specifically expressed as a percentage (%). tries in South America and Africa. This discussed in other articles. Epidemiology(2-4,7) is because iron deficiency accounts for Definition(1,2) 50% of this prevalence and it is closely Anemia is the most common hema- linked to nutritional deficiencies, the- The word anemia is of Greek origin, tological disorder in childhood. In refore, to the social and development meaning “without blood.” It is defined 2008, the World Health Organization conditions in these countries. as the reduction in the concentration (WHO) published the results of a sur- In addition to acknowledging iron of hemoglobin (Hb), erythrocyte mass vey of 192 member states, establishing deficiency as the most common cause of 214 EN - PEDIATRÍA INTEGRAL Pediatr Integral 2021; XXV (5): 214 – 221 ANEMIA. CLASSIFICATION AND DIAGNOSIS Table I. Normal values of the red series according to age and gender casians or Asians. HbS and HbC are (various sources) more common in black and Hispanic populations. Furthermore, within Age Hb (g/dl) Hct (%) MCV (fl) the same country there are areas with 1-3 days 19,5 58 98-118 a higher prevalence of hemoglobino- (14.5-235) (45-72) pathies, endocellular parasites, such 7 days 17,5 55 88-126 as malaria and infestation with intes- (14-22) (43-67) tinal parasites that impact on the 14 days 16,5 50 86-119 prevalence of anemia. Thus, thalas- (13-20) (42-66) semic syndromes are more prevalent 1 month 14 43 85-123 on the Mediterranean coast, a large (10-18) (31-51) part of Africa, the Middle East, the 2 months 11,5 35 77-118 Indian subcontinent, and Southeast (9-14) (28-42) Asia; conversely, G6PDH deficiency 3-12 months 11,5 35 74-108 is observed, predominantly, in mala- (9.5-13-5) (29-41) ria endemic areas, since it seems to 12-24 months 12,5 37 71-89 be a protective factor against this (11-14) (32-42) infection (a higher prevalence is 2-3 years 12,6 37 74-89 found among: Kurdish Jews, Sardi- (11-14.2) (33-41) nian, Nigerian, African-American, 4-6 years 12,9 38 77-91 Filipino and Greeks). (11.7-14,1) (34-42) • Height above sea level: the higher above the sea level, the higher the 7-10 years 13,5 40 78-91 (12-15) (35-45) Hb count, since lower oxygen con- tent in the air results in a stimulus 11-14 years Female 13,7 40 80-94 (12.3-15,1) (36-44) for hematopoiesis. 14,3 46 80-94 Pathophysiology(2,5) Male (12.6-16) (40-52) 15-18 years Female 13,7 40 81-96 Anemia is the result of the imbalance (11.5-15,9) (34-46) between production and loss of red blood 15,4 46 81-96 cells. The redistribution of blood, the sti- Male (13.7-17.1) (40-52) mulation of erythropoiesis and the decrease in the affinity of Hb for O2 are compensa- tory mechanisms. anemia in pediatric age worldwide, we - 3-6 months: iron deficiency is must take into account the factors and rare during this period, and he- Erythropoiesis mainly takes place causes of this disorder that influence its moglobinopathies must be ruled in the bone marrow during postnatal prevalence: out. and adult life (in the fetal period and • Age: Hb and Hct counts vary - 6 months-adolescence: there are up to 6 months of extrauterine life, the throughout childhood (Table I), differences in Hb counts accor- endodermal sinus also participates, as well as the causes of anemia ding to age and sex (Table I). where it begins at 3-4 weeks of gesta- differ according to the age of the During this entire stage, the main tion, and later on in the liver). Various patients: cause of anemia is iron deficiency. regulatory factors (being blood oxygen - Birth-3 months: Hb achieves • Sex: starting at puberty, testoste- saturation the main one) act on the peri- maximum concentrations (16.5- rone secretion induces an increase tubular cells of the kidneys involved in 18.5 g/dl) in the newborn and in erythrocyte mass, which is why the synthesis of erythropoietin (EPO), drops to 9-10 g/dl between 6-9 the normal level of Hb is higher in a hormone that acts on the hemato- weeks of life, as a consequence of men than in women. On the other poietic precursors of the bone marrow, increased oxygenation of tissues hand, some hereditary anemias which finally give rise to mature red and a drastic decrease in erythro- are X-linked, and hence, being blood cells. During this complex pro- poiesis; in what is referred to as more frequent in men (eg, glu- cess of differentiation and maturation “physiological anemia of infancy”. cose-6-phosphate dehydrogenase leading to the production of the mature Any anemia in this age group that (G6PDH) deficiency and sidero- erythrocyte, the participation of diffe- differs from the characteristics of blastic anemia). rent molecules, growth factors (G and physiological anemia (Hb <9 g/dl, • Race and ethnicity: normal Hb GM-CSF), trace elements (such as iron, anemia prior to 1 month of age, levels are observed with approxima- essential for the elaboration of the heme or signs of hemolysis) will require tely 0.5 g/dl less in black children group of Hb, copper and zinc) and cyto- further study. compared to those observed in Cau- kines (IL 1, 3, 4, 6, 9 and 11). PEDIATRÍA INTEGRAL - EN 215 ANEMIA. CLASSIFICATION AND DIAGNOSIS Medical history Mature erythrocytes are shaped determined by: adaptation mechanisms, like a biconcave disc, they are filled age of onset, underlying disease and An adequate medical history is the with Hb in the inside and are devoid type of onset (acute or chronic): starting point for the etiological diag- of mitochondria or other organelles. Hb • Pale skin and mucous membranes: nosis of anemia. In addition to noti- is composed of 4 globin subunits and direct consequence of the decrease cing the age, gender, ethnicity and heme groups, and it is involved in the in Hb and the accompanying peri- geographical origin of the patient, the exchange of oxygen and carbon dioxide pheral vasoconstriction. Sometimes following should be investigated: throughout the body. pallor may not be evident until the • Symptoms (see previous section): After erythrocytes have been in cir- Hb level falls below 8 g/dl and can beginning and speed of onset, tole- culation for a long period (half-life 120 be difficult to identify depending on rance, history of bleeding (digestive, days), they are taken up and destroyed the pigmentation of the skin.