Hemolytic Anemia Classification
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Hemolytic Anemia Classification

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What is a key diagnostic feature of hemolytic anemia?

Shortening of the life of red blood cells

What is the basis for classifying hemolytic anemia?

The cause of hemolysis, localization of hemolysis, and congenital or acquired character of the disease

What type of anemia is caused by defects in the plasmolemma of erythrocytes?

Membranopathy

What is an example of an enzymatic disorder of erythrocytes?

<p>Pyruvate kinase deficiency</p> Signup and view all the answers

What is an example of anemia caused by exoerythrocytic factors?

<p>Paroxysmal nocturnal hemoglobinuria</p> Signup and view all the answers

What is an example of an infectious disease that causes anemia?

<p>Malaria</p> Signup and view all the answers

What is the primary mechanism of anemia in individuals with vitamin B12 or folic acid deficiency?

<p>Disruption of DNA synthesis</p> Signup and view all the answers

Which of the following is a characteristic of megaloblastic anemia?

<p>Macrocytes and megalocytes</p> Signup and view all the answers

What is the likely consequence of atrophic gastritis in individuals with pernicious anemia?

<p>Increased risk of gastric cancer</p> Signup and view all the answers

Which of the following is a common feature of erythrocytes in megaloblastic anemia?

<p>Erythrocytes with remnants of the nucleus</p> Signup and view all the answers

What is the primary mechanism of anemia in individuals with iron deficiency?

<p>Disruption of heme synthesis</p> Signup and view all the answers

What is the term used to describe erythrocytes with an increased average volume containing the nucleus?

<p>Megaloblasts</p> Signup and view all the answers

What is the main cause of megaloblastic anemia?

<p>Insufficiency of vitamin B12 or folic acid</p> Signup and view all the answers

What is a common observation in the blood picture of megaloblastic anemia?

<p>Anisocytosis and macrocytosis</p> Signup and view all the answers

What is a notable feature of the bone marrow in megaloblastic anemia?

<p>Erythroid hyperplasia and megaloblastic changes</p> Signup and view all the answers

What is observed in the bone marrow of tubular bones in megaloblastic anemia?

<p>It becomes red and takes on the appearance of 'raspberry jelly'</p> Signup and view all the answers

What is a complication of megaloblastic anemia?

<p>Hemorrhagic syndrome</p> Signup and view all the answers

What is observed in neutrophils in megaloblastic anemia?

<p>Neutropenia and hypersegmented nuclei</p> Signup and view all the answers

What is a characteristic of the bone marrow in megaloblastic anemia?

<p>It takes on the appearance of 'raspberry jelly'</p> Signup and view all the answers

Which of the following is a complication of megaloblastic anemia?

<p>Atrophic glossitis</p> Signup and view all the answers

What is the primary cause of iron deficiency anemia?

<p>Chronic hemorrhage</p> Signup and view all the answers

What is a characteristic of the oral mucosa in megaloblastic anemia?

<p>It becomes atrophic and ulcerated</p> Signup and view all the answers

What is a consequence of impaired maturation of germ cells in both sexes?

<p>Infertility</p> Signup and view all the answers

What is a common cause of iron deficiency anemia in addition to chronic hemorrhage?

<p>Ancylostoma invasion</p> Signup and view all the answers

What is the primary cause of iron deficiency in cases of gluten enteropathy, achlorhydria, and after gastrectomy?

<p>Malabsorption of iron</p> Signup and view all the answers

What is the characteristic of erythrocytes in iron deficiency anemia?

<p>Microcytic and hypochromic</p> Signup and view all the answers

What is the term used to describe the condition characterized by furrowed, striped, and brittle nails, as well as atrophic glossitis and dysphagia?

<p>Plummer-Vinson syndrome</p> Signup and view all the answers

What is the consequence of atrophic gastritis in individuals with iron deficiency?

<p>Decreased iron absorption</p> Signup and view all the answers

What is the characteristic of erythrocytes in severe iron deficiency?

<p>Ring-shaped and ovalocytes</p> Signup and view all the answers

What is the relationship between iron deficiency and chronic hypoxia?

<p>Iron deficiency causes chronic hypoxia</p> Signup and view all the answers

What is the characteristic of acute leukemia?

<p>Proliferation of blast cells and malignant flow</p> Signup and view all the answers

Which of the following is a type of leukemia with a relative good prognosis?

<p>Chronic leukemia</p> Signup and view all the answers

What is the term used to describe leukemia with tens and hundreds of thousands of leukocytes per 1 mm3 of blood?

<p>Leukemic</p> Signup and view all the answers

What is the classification of leukemia based on the degree of maturity of tumor cells?

<p>By the degree of maturity of tumor cells</p> Signup and view all the answers

What is the characteristic of undifferentiated acute leukemia?

<p>Develops from the first three classes</p> Signup and view all the answers

What is the classification of chronic leukemia?

<p>By the histogenetic origin</p> Signup and view all the answers

What is the main difference between aplastic and hypoplastic anemia?

<p>Aplastic anemia is characterized by a complete absence of bone marrow cells, while hypoplastic anemia is characterized by a reduced number</p> Signup and view all the answers

What is the most common cause of acquired aplastic anemia?

<p>Idiopathic causes</p> Signup and view all the answers

What is the term used to describe the combination of anemia, neutropenia, lymphopenia, and thrombocytopenia?

<p>Pancytopenia</p> Signup and view all the answers

What is the role of autoreactive T lymphocytes in aplastic anemia?

<p>They play a role in the pathogenesis of bone marrow aplasia</p> Signup and view all the answers

What is the characteristic of the bone marrow in aplastic anemia?

<p>It is hypocellular</p> Signup and view all the answers

What is the consequence of infectious complications in aplastic anemia?

<p>Development of secondary immunodeficiency</p> Signup and view all the answers

What is a characteristic of the spleen in patients with acute monocytic leukemia?

<p>It is relatively small, weighing around 500-600 grams</p> Signup and view all the answers

What is the effect of neoplastic cells on the blood of patients with acute monocytic leukemia?

<p>A decrease in the number of other uniform elements</p> Signup and view all the answers

What is a consequence of anemia in patients with acute monocytic leukemia?

<p>Pallor of the skin and symptoms of hypoxia</p> Signup and view all the answers

What is the cause of petechial hemorrhages in patients with acute monocytic leukemia?

<p>Thrombocytopenia</p> Signup and view all the answers

What is a characteristic of disease in patients with promyeloblast leukemia?

<p>An increase in the permeability of the vascular wall</p> Signup and view all the answers

What is a consequence of leukemic infiltrates in patients with acute monocytic leukemia?

<p>Narrowing of the lumen of the vessels</p> Signup and view all the answers

What is the characteristic of the bone marrow in acute lymphoblastic leukemia (ALL)?

<p>It becomes juicy, crimson-red in color</p> Signup and view all the answers

What is the age range when acute myeloblastic leukemia (AML) is most commonly found?

<p>15-20 years</p> Signup and view all the answers

What is the characteristic of acute undifferentiated leukemia?

<p>Infiltration of all hematopoiesis and parenchymatous organs with undifferentiated homogeneous cells</p> Signup and view all the answers

What is the term used to describe the characteristic leukemia failure in acute leukemias?

<p>Hiatus leucemicus</p> Signup and view all the answers

What is the characteristic of the bone marrow in acute myeloblastic leukemia (AML)?

<p>It becomes greenish (pus-like) in color</p> Signup and view all the answers

What is the characteristic of acute leukemias in terms of cell proliferation?

<p>Proliferation of blasts without signs of differentiation</p> Signup and view all the answers

What is the average life expectancy of a patient with chronic leukemia without treatment?

<p>10-15 years</p> Signup and view all the answers

Which type of chronic leukemia is more common in people older than 60 years?

<p>Chronic lymphocytic leukemia (CLL)</p> Signup and view all the answers

What is the term used to describe the stage of chronic leukemia where blast forms of cells appear in the blood?

<p>Blast crisis</p> Signup and view all the answers

What is a characteristic of chronic myeloid leukemia (CML)?

<p>Occur in all age groups, but most often in 40-50 years</p> Signup and view all the answers

What is the term used to describe the group of diseases that includes chronic myeloid leukemia (CML), chronic monocytic leukemia, true polycythemia, myelofibrosis, and idiopathic thrombocythemia?

<p>Systemic myeloproliferative diseases</p> Signup and view all the answers

What is the characteristic of chronic lymphocytic leukemia (CLL)?

<p>More common in people older than 60 years</p> Signup and view all the answers

What type of cells are mainly represented in cases of leukemia infiltrates?

<p>Myelocytes and promyelocytes</p> Signup and view all the answers

What is the characteristic of the spleen in chronic myelocytic leukemia?

<p>It increases to 5-6 kg in weight</p> Signup and view all the answers

What is a common accompaniment of chronic lymphocytic leukemia?

<p>Autoimmune hemolytic anemia</p> Signup and view all the answers

What is the appearance of the bone marrow in chronic myelocytic leukemia?

<p>It becomes pyoid</p> Signup and view all the answers

What is the characteristic of the lymph nodes in chronic lymphocytic leukemia?

<p>They are significantly enlarged and soft</p> Signup and view all the answers

What is the average life expectancy of patients with chronic lymphocytic leukemia?

<p>3 to 7 years</p> Signup and view all the answers

What is a characteristic of true polycythemia?

<p>Decreased erythropoietin level and thrombocytosis</p> Signup and view all the answers

What is the cause of proliferation of fibroblasts in myelofibrosis?

<p>Tumor growth factor of thrombocytes and TGF-p</p> Signup and view all the answers

What is a characteristic of paraproteinemic leukemia?

<p>Production of homogeneous immunoglobulins or their fragments</p> Signup and view all the answers

What is a diagnostic feature of myeloma disease?

<p>Presence of Bens-Jones protein in urine</p> Signup and view all the answers

What is a possible complication of true polycythemia?

<p>Blast crisis</p> Signup and view all the answers

What is a characteristic of blood in myelofibrosis?

<p>Presence of precursors of granulocytes and erythrocytes of varying degrees of maturity</p> Signup and view all the answers

Study Notes

Hemolytic Anemia

  • Hemolytic anemia is a group of anemia characterized by the shortening of the life of red blood cells.

Classification of Hemolytic Anemia

  • Classification is based on three factors: cause of hemolysis, intra- or extravascular localization of hemolysis, and congenital or acquired character of the disease.

Anemias Caused by Endoerythrocytic Factors

  • Hereditary anemia:
    • Defects of the plasmolemma of erythrocytes (membranopathy):
      • Hereditary spherocytosis
      • Elliptocytosis
    • Enzymatic disorders of erythrocytes (fermentopathy):
      • Insufficiency of glucose-6-phosphate dehydrogenase
      • Pyruvate kinase deficiency
    • Disturbance of hemoglobin synthesis (hemoglobinopathy):
      • Disorders of globin chains (hemoglobin variants)
      • Reduction of synthesis of globin chains (thalassemia)
  • Acquired anemia:
    • Paroxysmal nocturnal hemoglobinuria

Anemias Caused by Exoerythrocyte Factors

  • Acquired anemia:
    • Autoimmune hemolytic anemia:
      • Thermal antibody
      • Cold-blooded
      • Anemia in autoimmune diseases
    • Immune hemolytic anemia:
      • Hemolytic disease of newborns
      • Transfusion hemolytic reaction
      • Medicinal immune hemolytic anemia
      • Hemolysis in response to toxins and chemicals
  • Mechanical damage of erythrocytes:
    • Microangiopathic anemia (erythrocyte fragmentation syndrome)
  • Anemia in infectious diseases:
    • Malaria
  • Hyperplenism
  • Mixed species

Classification of Diserythropoietic Anemia

  • Classification of diserythropoietic anemia is based on the stage of erythropoiesis affected
  • Two main categories: destruction of proliferation and differentiation of stem cells, and destruction of proliferation and differentiation of erythroblasts

Destruction of Proliferation and Differentiation of Stem Cells

  • Aplastic anemia: characterized by reduction or absence of hematopoietic stem cells
  • Aplasia of the erythroblastic sprout: failure of stem cells to differentiate into erythroblasts
  • Anemia in renal insufficiency: impaired erythropoiesis due to chronic kidney disease
  • Anemia in endocrine disorders: hormonal imbalances affecting erythropoiesis

Destruction of Proliferation and Differentiation of Erythroblasts

  • Disruption of DNA synthesis:
    • Insufficiency or impairment absorption of vitamin B12 and folic acid (megaloblastic anemia)
  • Destruction of Hb synthesis:
    • Violation of heme synthesis (Fe deficiency)
    • Violation of globin synthesis (thalassemia)
  • Unknown or mixed mechanisms:
    • Anemia due to a violation of Fe metabolism (anemia sideroblast, with chronic diseases, with myelodysplastic syndrome)

Megaloblastic Anemia

  • Characterized by violations of hemopoiesis, manifested in the appearance of megaloblasts during the differentiation of the myeloid germ
  • Common causes: insufficiency of either vitamin B12 or folic acid
  • Rare causes: cytotoxic effect of drugs that inhibit DNA synthesis
  • Blood picture:
    • Decrease in the number of erythrocytes, granulocytes, and platelets (pancytopenia)
    • Anisocytosis, macrocytosis, poikilocytosis
    • Nuclei and Howell-Jowley bodies appear in erythrocytes in advanced stages
    • Decrease in reticulocytes, neutropenia, and appearance of large neutrophils with hypersegmented nuclei
    • Fewer platelets, and possibly hemorrhagic syndrome
  • Bone marrow picture:
    • Red bone marrow of tubular bones resembling "raspberry jelly"
    • Erythroid hyperplasia, megaloblastic changes, retention of nuclei in differentiating cells, polyploidy, and nuclear fragmentation
    • Giant metamyelocytes, erythrophagia, and hemosiderosis appear
    • Hb synthesis is less disturbed

Bone Marrow Characteristics

  • Bone marrow of tubular bones appears as "raspberry jelly" due to red color
  • Erythroid hyperplasia, megaloblastic changes, retention of nuclei, polyploidy, and nuclear fragmentation observed
  • Giant metamyelocytes, erythrophagia, and hemosiderosis appear

Effects on Oral Mucosa and Gastrointestinal Tract

  • Atrophic glossitis (reduction and disappearance of tongue papillae) and ulceration of oral mucosa
  • Chronic atrophic gastritis, atrophy of small intestine villi, and infertility due to impaired germ cell maturation

Iron Deficiency Anemia

  • Main cause is chronic hemorrhage, particularly severe menstrual bleeding and latent hemorrhages
  • Ancylostoma invasion causes significant gastrointestinal bleeding and is a major cause of iron deficiency anemia
  • Deficiency can also be caused by malabsorption in gluten enteropathy, achlorhydria, and after gastrectomy

Blood Changes

  • Anemia is hypochromic, microcytic, and regenerative
  • Erythrocytes are pale, reduced in size, and have nuclear remains
  • Polychromatophilic erythrocytes and reticulocytes present
  • Irregular erythrocytes, including ringshaped, oval, and rod-shaped red blood cells

Bone Marrow Puncture

  • No iron-containing material in bone marrow punctate, giving a negative Berlin azure reaction
  • Erythroid hyperplasia, hypochromic and microcytic red blood cells

Other Organ and Tissue Changes

  • Iron deficiency leads to depletion of iron-containing enzymes and chronic hypoxia
  • Nail changes: furrowed, striped, brittle, and eventually spoon-shaped (coilonichia)
  • Atrophic glossitis, cracks in mouth corners, and dysphagia (swallowing disorders) due to mucous membrane folding in the cervical esophagus
  • Atrophic gastritis leads to achlorhydria, intensifying iron deficiency

Aplastic and Hypoplastic Anemia

  • Aplastic anemia is characterized by a small or absent number of cells in the bone marrow.
  • Hypoplastic anemia is characterized by a reduced number of cells in the bone marrow.
  • Congenital aplastic anemia is also known as Fanconi anemia and is often accompanied by other anomalies such as bone defects or congenital heart diseases.
  • Acquired aplastic anemia can be caused by:
    • Idiopathic anemia (50%)
    • Irradiation
    • Treatment with massive doses of cytotoxic drugs
    • Use of certain drugs (e.g. levomycetin, sulfonamides) or idiosyncrasy to medicines
    • Viral infections (e.g. cytomegalovirus or Epstein-Barr virus)
    • Autoreactive T lymphocytes may also play a role in the pathogenesis of bone marrow aplasia
  • The main manifestation of aplastic anemia is the inadequacy of proliferation and differentiation of hematopoietic stem cells, leading to pancytopenia, immunodeficiency, and a tendency to bleeding.

Classification of Tumors of the Blood System

  • Primary systemic leukemia: tumor cells grow in the bone marrow and often enter the blood.
  • Primary-local lymphomas: tumor cells grow in extraosternal lymphoid tissue.
  • Leukemia classification:
    • By degree of increase in blood:
      • Leukemic (tens to hundreds of thousands of leukocytes per 1 mm3 of blood)
      • Subleukemic (no more than 15,000-25,000 in 1 mm3 of blood)
      • Leukopenic (the number of leukocytes is reduced, but leukemic cells are detected)
      • Aleukemic (leukemia cells in the blood are absent)
    • By degree of maturity of tumor cells and nature of the course:
      • Acute leukemia: proliferation of blast cells and malignant flow
      • Chronic leukemia: proliferation of differentiated leukemia cells and relative goodness of the course
    • Histogenetic forms of acute leukemia:
      • Undifferentiated
      • Myeloblast
      • Lymphoblastic
      • Monoblast (myelomonoblast)
      • Erythromyeloblast
      • Megakaryoblast
    • Histogenetic forms of chronic leukemia:
      • Chronic leukemia of myelocytic origin:
        • Chronic myeloid leukemia
        • Chronic erythromyelosis
        • Erythremia
        • True polycythemia
      • Chronic leukemias of the lymphocytic series:
        • Chronic lymphocytic leukemia
        • Skin lymphoma (Cesari's disease)
        • Paraproteinemic leukemia (myeloma, or plasmacytoma, Waldenstrom's primary macroglobulinemia, Franklin heavy chain disease)
      • Chronic leukemias of monocytic origin:
        • Monocytic (myelomonocytic) leukemia
        • Histiocytosis (histiocytosis X)

Hemoblastoses

  • Hemoblastoses are tumors of the hematopoietic and lymphatic tissue.
  • Leukemia is a systemic tumor lesion of the hematopoietic tissue, while malignant lymphomas are regional tumor lesions of lymphoid tissue with possible generalization.

Acute Leukemia

  • Characterized by acute onset (high fever, severe intoxication) and rapid progression of the disease.
  • The most frequent forms of acute leukemia are acute lymphoblastic leukemia (ALL) and acute myeloblastic leukemia (AML).

Acute Lymphoblastic Leukemia (ALL)

  • Mainly found in early childhood (3-4 years).
  • Bone marrow becomes juicy, crimson-red, and resembles a crimson jelly.

Acute Myeloblastic Leukemia (AML)

  • Occurs at any age, but most often in adolescents (peak at 15-20 years).
  • Bone marrow acquires a greenish (pus-like) hue, also known as pioid marrow.

Common Characteristics of Acute Leukemias

  • Proliferation of blasts without signs of differentiation.
  • Infiltration of all hematopoiesis and parenchymatous organs with undifferentiated homogeneous cells.
  • Leukocytosis with the presence of blasts in peripheral blood.
  • Characteristic leukemia failure (hiatus leucemicus) - a sharp increase in blast cells and single mature elements in the absence of transient maturing forms.

Clinical Manifestations

  • Lymph node enlargement is often observed with ALL and acute monocytic leukemia, but usually absent with AML.
  • Enlargement of the spleen is relatively small, reaching 500-600 grams.
  • Anemia is severe and rapidly developing, manifested by pallor of the skin and symptoms of hypoxia.
  • Thrombocytopenia leads to petechial hemorrhages (hemorrhagic diathesis).
  • Neutropenia manifests as frequent infectious diseases and ulceration (necrosis) on the mucous membranes of the oral cavity, tonsils, and gastrointestinal tract.
  • Reduction of immunity and general reactivity of the organism is accompanied by activation of infections and development of alterative inflammation.

Chronic Leukemia

  • Characterized by a gradual onset, slow progression, and a life expectancy of 10-15 years without treatment.

Chronic Lymphocytic Leukemia (CLL)

  • More common in people older than 60 years
  • Leukemia cells are represented by lymphoid cells of B-lymphocyte origin
  • Unable to differentiate into antibody-producing cells
  • Diffuse leukemia infiltration of the bone marrow, spleen, liver, and lymph nodes
  • Often accompanied by autoimmune hemolytic anemia
  • Average life expectancy of patients is 3 to 7 years, and treatment does not have a significant impact on it
  • Spleen increases to 1 kg

Chronic Myelocytic Leukemia (CML)

  • Occurs in all age groups, but most often in 40-50 years
  • Leukemia cells are represented by myeloid cells - myelocytes, promyelocytes
  • Often, the Philadelphia chromosome is identified: deletion of the 22nd chromosome due to reciprocal translocation between the 9th and 22 chromosomes
  • Forming a hybrid bcr-abl gene responsible for the tumor transformation in this leukemia
  • Bone marrow of flat and tubular bones acquires a pyoid appearance (pus-like)
  • Spleen increases to 5-6 kg, with a tense capsule, gray-red in color with a brownish tint on the cut
  • Small areas of white necrosis are visible under the capsule

Blast Crisis

  • Develops in the terminal stage of chronic leukemia
  • Blast forms of cells appear in the blood
  • Disease sharply becomes heavier, and the current resembles acute leukemia
  • Often ends in a fatal outcome

True Polycythemia

  • Characterized by significant erythrocytosis, moderate granulocytosis, and thrombocytosis
  • Splenomegaly and decreased erythropoietin level are present
  • Tendency to thrombotic and thrombotic hemorrhagic complications
  • Blast crisis possible in the terminal stage

Myelofibrosis

  • Growth of fibrous tissue in bone marrow cavities
  • Megakaryocytosis of bone marrow, leading to thrombocytosis
  • Tumor growth factor of thrombocytes and TGF-p may cause fibroblast proliferation
  • Anemia, poikilocytosis, and massive splenomegaly are expressed
  • Blood precursors of granulocytes and erythrocytes of varying degrees of maturity are found
  • Blast crisis possible

Paraproteinemic Leukemia

  • Characterized by the ability of leukemia cells to synthesize homogeneous immunoglobulins or their fragments (paraproteins)
  • Paraproteins form an M-gradient during plasma protein electrophoresis
  • Allocate the following forms: myeloma, Waldenstrom's macroglobulinemia, Franklin heavy chain disease

Myeloma (Rustitsky-Kahler disease)

  • Leukemic cells are B-lymphocytes and plasma cells producing Ig of one class (IgG, IgA, or light chains)
  • Myeloma cells produce Ig that are filtered into urine and called "Bens-Jones protein"
  • Myeloma cells are not detected in peripheral blood (aleukemic leukemia)
  • Proliferating myeloma cells in bone marrow produce a factor that activates osteoclasts, leading to:
    • Foci of axillary resorption and osteoporosis
    • Pain in bones and frequent spontaneous fractures
    • Osteolysis leading to hypercalcemia and focal points of metastatic calcification

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Learn about the classification of hemolytic anemia, including the causes of hemolysis, localization of hemolysis, and congenital or acquired character of the disease.

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