Anemia Hemolítica

Epidemiologia e Etiologia

Epidemiologia

• 5% de todos os casos de anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types
• Causas hereditárias presentes nos primeiros anos de vida.
• A anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica autoimune (AHAI) é mais MAIS Androgen Insensitivity Syndrome comum nos adultos de meia-idade e mais MAIS Androgen Insensitivity Syndrome velhos.
• O sexo masculino é predominantemente afectado pela esferocitose hereditária, uma vez que está ligada ao cromossoma X.

Causas

Hemólise intravascular

• Mecânica (válvulas protésicas mecânicas, hemodiálise)
• Reação transfusional
• Hemoglobinúria paroxística noturna (HPN)
• Infeção (septicemia)
• Microangiopática (síndrome hemolítico urémico, púrpura trombocitopénica trombótica, coagulação intravascular disseminada)

Hemólise extravascular

• Anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica intrínseca (estrutura anormal da membrana ou forma dos GV)
  • Defeitos na membrana:
    • Esferocitose hereditária
  • Deficiência enzimática:
    • Deficiência de G6PD G6PD Pentose Phosphate Pathway
    • Défica da piruvato desidrogenase
  • Hemoglobinopatia:
    • Anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types falciforme
    • Talassemia
• Anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica extrínseca (GV revestidos)
  • AHAI
    • Anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types por aglutininas frias
    • Anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types por aglutininas quentes
  • Incompatibilidade ABO, incompatibilidade Rhesus
  • Fármacos (por exemplo, rifampicina, anti-inflamatórios não esteróides, penicilina)
  • Infeções (malária), gangrena gasosa
  • Hiperesplenismo

Fisiopatologia

A anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica é uma anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types normocítica.

• Hemólise extravascular
  • A degradação normal dos GV ocorre no baço a cada 120 dias.
  • Ocorre um aumento da clearance esplênica:
    • Marcação dos GV com IgG IgG The major immunoglobulin isotype class in normal human serum. There are several isotype subclasses of igg, for example, igg1, igg2a, and igg2b. Hypersensitivity Pneumonitis/ IgM IgM A class of immunoglobulin bearing mu chains (immunoglobulin mu-chains). Igm can fix complement. The name comes from its high molecular weight and originally being called a macroglobulin. Immunoglobulins: Types and Functions em condições autoimunes
    • GV com defeito intrínseco: anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types falciforme, esferocitose, deficiência de G6PD G6PD Pentose Phosphate Pathway
  • O aumento da degradação dos GV resulta num aumento do grupo heme em circulação, que é convertido em bilirrubina.
  • Bilirrubina em excesso→ bilirrubinemia e icterícia não conjugada
  • O ferro resultante da degradação também se acumula no baço.
  • O excesso de funcionamento e de clearance resulta em esplenomegalia.
• Hemólise intravascular
  • Os GV fragmentam-se ao passar pelo lúmen do capilar (microangiopático) ou por válvulas protésicas (macroangiopático).
  • A fragmentação dos GV resulta em na presença esquizócitos no esfregaço.
  • A hemoglobina no sangue liga-se à haptoglobina.
  • O heme em excesso também se apresenta na urina como hematúria/hemoglobinúria.

Manifestações Clínicas

• Icterícia
• Esplenomegalia (principalmente em causas extravasculares)
• Colelitíase
• Hematúria/hemoglobinúria
• Sintomas de anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types:
  • Dispneia
  • Fadiga
  • Síncope
  • Taquicardia
• Pacientes com anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types falciforme apresentam síndromes de dor aguda.
• Pacientes com deficiência de G6PD G6PD Pentose Phosphate Pathway e pacientes com HPN apresentam-se com hematúria episódica.
• Os pacientes com AHAI (fria) apresentam com sensibilidade ao frio.

Diagnósticos

Geral

• ↓ Hb Hb The oxygen-carrying proteins of erythrocytes. They are found in all vertebrates and some invertebrates. The number of globin subunits in the hemoglobin quaternary structure differs between species. Structures range from monomeric to a variety of multimeric arrangements. Gas Exchange, N-VCM, N-HCM
• Reticulocitose (≤ 3%)
• Diminuição do hematócrito
• Aumento da desidrogenase láctica
• Diminuição da haptoglobina
• Aumento da bilirrubina não conjugada

Hemólise intravascular

• Hemoglobinúria/hemossiderinúria: hemoglobina que excede a ligação à haptoglobina ( mais MAIS Androgen Insensitivity Syndrome sensível)
• Esquizócitos no esfregaço de sangue
• Teste de Coombs Indireto (positivo para complementos)

Hemólise extravascular

• Teste de Coombs direto (positivo para IgM IgM A class of immunoglobulin bearing mu chains (immunoglobulin mu-chains). Igm can fix complement. The name comes from its high molecular weight and originally being called a macroglobulin. Immunoglobulins: Types and Functions/IgGG)
• Esfregaço sanguíneo: esferócitos, anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types falciforme, aglutinação (em IgM IgM A class of immunoglobulin bearing mu chains (immunoglobulin mu-chains). Igm can fix complement. The name comes from its high molecular weight and originally being called a macroglobulin. Immunoglobulins: Types and Functions AHAI)

Tratamento

• Tratar a causa subjacente.
• Suplementação com ácido fólico para aumentar a eritrocitose compensatória.
• Tratar a hipercalemia pelo aumento da degradação celular.
• Esplenectomia no caso das doenças hemolíticas extravasculares

Diagnósticos Diferenciais

• Anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types falciforme: uma hemoglobinopatia hereditária causada por uma deficiência na cadeia de beta-globina. É uma doença autossómica recessiva e apresenta sintomas de hemólise em condições hipóxicas.
• Talassemia: hemoglobinopatia hereditária causada por uma anormalidade na cadeia alfa ou beta-globina. Resulta num conjunto de sintomas que dependem em quais e quantos genes Genes A category of nucleic acid sequences that function as units of heredity and which code for the basic instructions for the development, reproduction, and maintenance of organisms. DNA Types and Structure estão afetados. Os pacientes têm hemólise crónica e apresentam sintomas de anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica, tornando-os dependentes de transfusões.
• Deficiência de G6PD G6PD Pentose Phosphate Pathway: uma deficiência enzimática da atividade da glicose-6-fosfato desidrogenase. Os pacientes apresentam hemólise episódica devido à exposição a determinados agentes de stress oxidativo.
• Esferocitose hereditária: uma deficiência hereditária em proteínas do citoesqueleto da membrana que estabilizam o GV. A deficiência resulta na perda da forma biconcava, passando para a forma esférica.
• Síndrome hemolítico urémico: uma tríade de hemólise, insuficiência renal e trombocitopenia, geralmente secundária a infeção por Escherichia coli Escherichia coli The gram-negative bacterium Escherichia coli is a key component of the human gut microbiota. Most strains of E. coli are avirulent, but occasionally they escape the GI tract, infecting the urinary tract and other sites. Less common strains of E. coli are able to cause disease within the GI tract, most commonly presenting as abdominal pain and diarrhea. Escherichia coli (O157:H7). A hemólise microangiopática é devida à aderência plaquetária às paredes vasculares.
• Coagulopatia intravascular disseminada: um desequilíbrio de plasmina e trombina leva à coagulação intravascular. Há um consumo excessivo de plaquetas, fatores de coagulação e fibrinogênio, pela formação de trombos. A trombose resulta na fragmentação das hemácias.
• Esplenomegalia: O aumento do baço pelo aumento da função é visto em anemias hemolíticas. Existem muitas outras causas de esplenomegalia: infiltração, fluxo sanguíneo anormal e distúrbios imunológicos.