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Pallor

David T. Teachey, MD

Pallor - BASICS

Pallor - description

  • Pallor is defined as paleness of the skin and may be a reflection of anemia or poor peripheral perfusion.
  • The normal range for hemoglobin is age dependent.
  • Anemia can be defined functionally as the inability of hemoglobin to meet cellular oxygen demand.
  • Parents often fail to notice pallor of gradual onset.
    • Grandparents or others who see child less often may be the 1st to suspect pallor.

Pallor - risk factors

  • Age between 6 months and 3 years, or adolescent females:
    • Peak age ranges for iron deficiency
  • Sex:
    • Some red cell–enzyme X-linked defects, such as glucose-6-phosphate dehydrogenase (G6PD) and phosphoglycerate kinase deficiencies are sex linked.
  • Race:
    • Black: Hemoglobins S and C, α- and β-thalassemia trait, G6PD deficiency
    • Southeast Asian: Hemoglobin E and α-thalassemia
    • Mediterranean descent: β-thalassemia and G6PD deficiency

Pallor - genetics

Familial history:

  • Some of the congenital hemolytic anemias are autosomal dominant.

Pallor - DIAGNOSIS

  • Determine 1st that the child appears pale, not simply fair skinned. 2nd, decide if there is a medical emergency associated with circulatory failure. If not, the goal is to investigate the etiology and intervene appropriately.
  • Phase 1: Assess for signs of shock.
    • If present, initiate emergency procedures as required to stabilize the patient, such as airway, breathing, and circulation.
  • Phase 2: If patient is stable, perform history, physical examination, and CBC with reticulocyte count to establish time of onset of pallor, associated symptoms, and level of anemia.
  • Phase 3: Specific diagnostic workup based on findings in phase 2.

Pallor - signs & symptoms

  • Pallor
  • Other signs and symptoms dependent on etiology

Pallor - history

  • Acute versus chronic onset:
    • Helps with differential diagnosis.
  • Associated symptoms: Weight loss, fever, night sweats, cough, and/or bone pain:
    • Suggest an underlying systemic illness, such as leukemia, infection, or rheumatologic disorder
  • Jaundice, scleral icterus, dark urine:
    • Suggest hemolysis
  • Age <6 months:
    • May represent a congenital anemia or isoimmunization
  • Premature infant:
    • Increased risk of both iron and vitamin E deficiency.
    • Exaggerated hyperbilirubinemia can be the presenting symptom of isoimmune hemolytic or other congenital hemolytic anemia.
  • Pica:
    • Often associated with plumbism and iron deficiency
  • Medications:
    • Can cause bone marrow suppression and/or hemolysis
  • Milk intake:
    • Cow’s milk <12 months of age and high milk intake are associated with iron deficiency.
  • Recent trauma and/or surgery:
    • Blood loss can result in iron deficiency.
  • Recent infection:
    • Can be associated with hemolysis or bone marrow suppression
    • Most common form of mild anemia in childhood
  • Family history:
    • Familial history of splenectomy and/or early cholecystectomy can be a clue for a previously undiagnosed hemolytic anemia.

Pallor - physical exam

  • Rapid respiratory rate, decreased BP, weak pulses, slow capillary refill
    • Indications of uncompensated anemia and/or shock
  • Frontal bossing and prominence of the malar and maxillary bones:
    • Extramedullary erythropoiesis
  • Enlarged spleen:
    • Hemolytic anemias, malignancy, infection
  • Glossitis:
    • Vitamin B
    • Scleral icterus or jaundice:
      • May indicate hemolysis
    • Systolic flow murmur:
      • Anemia
    • Bruits:
      • May indicate vascular malformations
    • Petechiae and bruising:
      • May indicate an associated thrombocytopenia, coagulopathy, or vasculitis
    • Dysmorphic features:
      • Diamond-Blackfan and Fanconi anemia are associated with other congenital defects, including thumb abnormalities, short stature, and congenital heart disease.

    Pallor - tests

    Pallor - lab

    • CBC with red cell indices:
      • Establishes the diagnosis of anemia, distinguishes by size: Normocytic, macrocytic, microcytic
    • Reticulocyte count:
      • Distinguishes between decreased production and increased destruction of red cells
    • Coombs test and antibody screen:
      • Identifies immune-mediated red cell destruction
      • Can have false positives and negatives
    • Peripheral blood smear:
      • Specific morphologic findings can be diagnostic.
    • Iron studies: Iron-binding capacity, serum iron, ferritin, transferrin
      • Iron-deficiency anemia or anemia of chronic disease
    • Hemoglobin electrophoresis with quantification:
      • Hemoglobinopathy
    • Lead studies: Serum lead, free erythrocyte protoporphyrin:
      • Plumbism
    • Stool guaiac:
      • Occult blood loss
    • Osmotic fragility:
      • Red cell membrane defects (spherocytosis)
      • Any spherocytic anemia may be positive
    • Quantitative red cell–enzyme assays:
      • Inherited RBC enzyme deficiencies
    • Serum folate, RBC folate, and serum vitamin BDeficiency
  • Pallor - diag proced-surgery

    Bone marrow aspiration and biopsy:

    • Malignancy or bone marrow failure syndrome

    Pallor - differencial diagnosis

    • Congenital:
      • Hemoglobinopathies: Sickle cell syndromes, thalassemia syndromes, other unstable hemoglobins
      • Erythrocyte membrane defects: Hereditary spherocytosis, elliptocytosis, stomatocytosis, pyropoikilocytosis, infantile pyknocytosis
      • Erythrocyte enzyme defects: G6PD deficiency, pyruvate kinase deficiency
      • Diamond-Blackfan anemia: Congenital pure red cell aplasia (rare)
      • Fanconi anemia: Constellation of varied cytopenias, multiple congenital anomalies, abnormal bone marrow chromosomal fragility
    • Infectious:
      • Septic shock
      • Can get mild anemia after mild infections in childhood (anemia of inflammation)
      • Infection-related bone marrow suppression: Parvovirus B19 infection
      • Infection-related hemolytic anemias: Epstein-Barr virus, influenza, Coxsackievirus, varicella, cytomegalovirus, Escherichia coli, Pneumococcus species, Streptococcus species, Salmonella typhi, Mycoplasma species
    • Nutritional/Toxic/Drugs:
      • Iron-deficiency anemia: Common cause of anemia in children, especially those <3 years of age and in female adolescents
      • Plumbism: Anemia usually due to coexisting iron deficiency. Very high lead levels associated with altered heme synthesis
      • Vitamin BMedication-induced bone marrow suppression: Chemotherapy; antibiotics, especially trimethoprim–sulfamethoxazole
      • Drug-related hemolytic anemia: Antibiotics, antiepileptics, azathioprine, isoniazid, nonsteroidal anti-inflammatory drugs
    • Trauma:
      • Acute blood loss
    • Tumor:
      • Leukemia with bone marrow infiltration
      • Metastatic tumors with bone marrow infiltration
    • Genetic/Metabolic:
      • Metabolic derangements: Severe electrolyte disturbance, pH disturbance, inborn errors
      • Schwachmann–Diamond syndrome: Marrow hypoplasia with associated pancreatic insufficiency and associated failure to thrive
    • Other:
      • Transient erythroblastopenia of childhood: Acquired pure RBC aplasia
      • Aplastic anemia: Bone marrow failure syndrome with at least 2 of the 3 blood cell lines eventually affected
      • Systemic diseases: Anemia of chronic disease, chronic renal disease, uremia
      • Hypothyroidism
      • Sideroblastic anemia: Defective iron utilization within the developing erythrocytes
      • Autoimmune and isoimmune hemolytic anemias
      • Microangiopathic hemolytic anemias: Thrombotic thrombocytopenic purpura (TTP), hemolytic uremic syndrome (HUS), disseminated intravascular coagulation (DIC)
      • Mechanical destruction: Vascular malformation, abnormal or prosthetic cardiac valves

    Pallor - TREATMENT

    Pallor - initial stabilization

    • Severe anemia of unclear etiology with hemodynamic instability:
      • Transfuse with packed RBCs cautiously.
      • In an autoimmune hemolytic process, the child is at risk for a transfusion reaction and there may be delay in obtainingcrossmatched blood
      • Obtain blood for diagnostic studies before transfusion if possible.
    • Circulatory failure without anemia:
      • Requires intensive monitoring and access to critical care in an emergency department or intensive care unit
      • Fluid resuscitation and/or inotropic pressor support as needed
    • Acute blood loss:
      • Treat circulatory failure as described.
      • Transfuse with packed RBCs, platelets, and fresh frozen plasma as needed.
    • Malignancies:
      • Emergency care should be directed toward treatment of circulatory failure and possible associated infection, and then to rapid diagnosis and treatment of the malignancy.
      • Consultation with an oncologist should be sought as soon as possible.

    Pallor - general measures

    • Treat underlying cause
    • Consider packed RBC transfusion if in extremis or severe anemia and low likelihood of recovery in near future
    • Consider emergent plasmapheresis if microangiopathic hemolytic anemia
    • Consider immunosuppresive medications (corticosteroids, intravenous immunoglobulin (IVIgG) if autoimmune hemolytic anemia
    • Iron-deficiency anemia:
      • Elemental iron

    Pallor - medication

    Elemental iron:

    • 4–6 mg/kg divided b.i.d.–t.i.d.
    • Absorbed best with acidic drinks, including orange juice; dairy products decrease absorption.
    • Reticulocyte should improve 72 hours after starting iron therapy; the hemoglobin may take a week to rise.
    • Iron should be continued for at least 3 months to replenish iron stores.

    Pallor - FOLLOW UP

    Based on determination of etiology

    Pallor - disposition

    Pallor - issues for referral

    • Severe or unexplained anemia
    • Anemias other than dietary iron deficiency or thalassemia trait
    • Recurrent iron deficiency
      • May suggest ongoing bleeding or iron malabsorption.
    • All bone marrow failure or infiltrative processes

    Pallor - bibliography

    1. Glader BE. Hemolytic anemia in children. Clin Lab Med. 1999;19:87–111.
    2. Graham EA. The changing face of anemia in infancy. Pediatr Rev. 1995;15:175–183.
    3. Monzon CM, Beaver D, Dillon TD. Evaluation of erythrocyte disorders with mean corpuscular volume (MCV) and red cell distribution width (RDW). Clin Pediatr. 1987;26:632–638.
    4. Segal G, Hirsh M, Feig S. Managing anemia in pediatric office practice, 1. Pediatr Rev. 2002;23;75–84.
    5. Sills RH. Indications for bone marrow examination. Pediatr Rev. 1995;16:226–228.

    Pallor - CODES

    Pallor - icd9

    783.61 Pallor

    >>>

    Book Source Details

    • Book Title: The 5-Minute Pediatric Consult
    • Author(s): M. William Schwartz MD; et al.
    • Year of Publication: 2008
    • Copyright Details: The 5-Minute Pediatric Consult, Copyright © 2008 Lippincott Williams & Wilkins.

    Other Book Chapters Related to Paleness

    Read excerpts from these other book chapters related to Paleness:

    Medical Books Excerpts
    • Pallor
    • "In A Page: Pediatric Signs and Symptoms" (2007)
    • Pallor
    • "Handbook of Signs & Symptoms (Third Edition)" (2006)
    • Skin, clammy
    • "Professional Guide to Signs & Symptoms (Fifth Edition)" (2006)
    • Pallor
    • "Professional Guide to Signs & Symptoms (Fifth Edition)" (2006)
    • Skin, clammy
    • "Alarming Signs and Symptoms: Lippincott Manual of Nursing Practice Series" (2007)
    • Pallor
    • "Alarming Signs and Symptoms: Lippincott Manual of Nursing Practice Series" (2007)
    • Skin, clammy
    • "Signs & Symptoms: A 2-in-1 Reference for Nurses" (2007)
    • Pallor
    • "Signs & Symptoms: A 2-in-1 Reference for Nurses" (2007)
    • Pallor (Anemia)
    • "The Diagnostic Approach to Symptoms and Signs in Pediatrics" (2006)
    • Pallor
    • "Nursing: Interpreting Signs and Symptoms" (2007)
    • Pallor
    • "Pediatric Complaints and Diagnostic Dilemmas" (2003)
    • Pallor
    • "The 5-Minute Pediatric Consult" (2008)
     

    Copyright Details: The 5-Minute Pediatric Consult, Copyright © 2008 Williams & Wilkins.

    More About Causes of Paleness




    More About This Book:
    Title: The 5-Minute Pediatric Consult
    Authors: M. William Schwartz MD; et al.
    Publisher: Lippincott Williams & Wilkins
    Copyright: 2008
    ISBN: 0-7817-7577-9

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