Yibian
 Shen Yaozi 
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diseaseInfantile Jacob's Syndrome
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bubble_chart Overview

Von Jaksch's syndrome, also known as von Jaksch's anemia, infantile nutritional-infectious anemia, or pseudoleukemia, is a type of anemia. The disease primarily affects infants aged 6 months to 2 years. The main causes include nutritional deficiencies (primarily lack of hematopoietic substances), rickets, and chronic or recurrent infections (such as respiratory infections, urinary tract infections, and suppurative skin infections). The clinical manifestations mainly involve nutritional anemia and extramedullary hematopoiesis, such as hepatosplenomegaly. The prognosis is generally good, with recovery possible after eliminating the causes, improving nutrition, and treating the anemia.

bubble_chart Clinical Manifestations

  1. Onset in infants and young children.
  2. History of chronic or recurrent infections.
  3. Slow onset, complexion gradually becomes pale or waxy yellow, emaciated, listless, often accompanied by irregular fever.
  4. Physical examination reveals significant hepatosplenomegaly, especially splenomegaly, which may extend to the pelvis and feels firm. Generalized lymph nodes may show grade I enlargement, and sometimes skin petechiae or edema may be observed.

bubble_chart Auxiliary Examination

  1. Blood picture Mostly manifests as grade II or above nutritional mixed anemia. The red blood cells vary in size, with frequent presence of abnormal shapes, polychromasia, and nucleated red blood cells; reticulocytes show grade I increase. White blood cells are increased, even reaching 30×109/l to 40×109/l, with various stages of immature granulocytes visible in the differential count (even a few myeloblasts may be seen), but the majority are still relatively mature cells. Platelets are generally normal, though a few cases may show a decrease.
  2. Bone marrow picture Hyperactive or markedly hyperactive. Cell classification and morphology are similar to those of nutritional mixed anemia. A few cases may show hypoplasia, with relative lymphocytosis and megakaryocyte reduction.

bubble_chart Treatment Measures

﹝Treatment﹞

(1) Control infection and eliminate infectious foci. (2) Strengthen nutrition and provide anti-rickets treatment.

(3) Treat as nutritional mixed anemia, administer iron supplements, vitamin B12, folic acid, etc.

(4) For severe cases, blood transfusion may be administered when necessary.

bubble_chart Differentiation

(1) Leukemia Acute leukemia progresses rapidly, often with a tendency to hemorrhage. The blood smear shows immature cells predominantly at the blast stage, and platelets are usually significantly reduced. The bone marrow exhibits typical leukemic changes. In infantile chronic myeloid leukemia, the blood and bone marrow show prominent granulocytic changes, and fetal hemoglobin is often significantly elevated. These characteristics can aid in differentiation.

(2) Leukemoid reaction The primary infectious focus can often be identified, splenomegaly is mild, and the blood smear lacks nucleated red blood cells. Granulocytes show signs of infection or toxicity, and the blood picture returns to normal after the primary disease is controlled.

(3) Other diseases with extramedullary hematopoiesis Conditions such as infantile osteopetrosis and myelofibrosis also present with anemia, splenomegaly, and immature granulocytes and erythrocytes in the peripheral blood. However, bone marrow aspiration is often unsuccessful. Bone marrow biopsy or skeletal X-rays can assist in differentiation.

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