Hematologic and Immunologic Emergencies

Chapter 23 Hematologic and Immunologic Emergencies



C.J. Carringer, Belinda B. Hammond


The number of patients presenting to emergency departments (EDs) with hematologic and immunologic emergencies is increasing because of better treatment and enhanced longevity of persons with these conditions. Several pathologic processes directly affect the blood and the organs that produce blood. Three problems commonly encountered by emergency nurses are anemia, sickle cell disease and crisis, and hemophilia. Patients with leukemia and lymphoma experience specific immunologic emergencies related to their disease and its treatment. These complications must be identified and managed. As the result of the dramatic increase in transplant survival, a growing number of organ recipients are being evaluated in EDs for transplant-related complications. Likewise, patients who are human immunodeficiency virus (HIV) positive have an increasing life expectancy and may present with a variety of clinical problems both related and unrelated to their HIV status.



Hematologic Emergencies



Anemia


Anemia exists when there is a deficiency in the number of red blood cells (RBCs) and thus a deficiency in the oxygen-carrying ability of the blood. Anemia can be acute or chronic and results from one of three possible mechanisms:



• Excessive blood loss


• Decreased production of RBCs


• Destruction of RBCs


Management of anemia depends on the acuity of onset and the severity of clinical symptoms. If anemia is suspected, the patient should be assessed for any of the following risk factors:



• Kidney disease


• Diabetes


• Cancer or cancer therapy


• Family history of anemia


• Chronic infection or disease


• Injury


• Anticoagulation therapy


• Gastrointestinal bleeding or malabsorption syndromes


• Abnormal vaginal bleeding


• Medications that suppress bone marrow function



Signs and Symptoms




• Fatigue, decreased energy level


• Dyspnea on exertion


• Chest pain if concurrent coronary artery disease


• Dizziness


• Pallor


• Complaints of “feeling cold”


• Headache



Diagnostic Procedures




• Complete blood count (CBC) with differential and peripheral smear. Anemia is defined as:


• Hematocrit of less than 39% in men and less than 36% in nonpregnant women

• Hemoglobin level less than 13.5 g/dL in men and less than 12 g/dL in women

• Coagulation studies if indicated


• Stool sample for occult blood


• Coagulation panel


• Endoscopy or colonoscopy to detect possible gastrointestinal bleeding



With acute blood loss, hemoglobin and hematocrit values remain normal until hemodilution occurs. Therefore these are poor early indicators of acute bleeding.



Therapeutic Interventions




• The primary goal of anemia management is to identify the underlying cause.


• If blood loss is acute, measures are aimed at stopping the bleeding. Specific interventions will depend on the source of bleeding.


• Administer supplemental oxygen.


• Anticipate possible need for intravenous volume replacement, type and crossmatching, and blood transfusion.


• Instruct patient to avoid nonsteroidal anti-inflammatory drugs (NSAIDs) and aspirin as these medications may cause or exacerbate gastrointestinal bleeding.


• Arrange outpatient follow up for the patient.



Anemia in the elderly is common—but it is not normal or harmless. Its causes are multifactorial, with nutritional deficiency accounting for one third of the causes and chronic illness responsible for another third.1



Sickle Cell Disease


Sickle cell disease is a congenital hemolytic anemia that occurs primarily, but not exclusively, in those of West African descent. Defective hemoglobin molecules cause RBCs to assume a sickled configuration instead of their usual “jelly doughnut” shape.


An individual with one sickle cell gene possesses the sickle cell trait, but the disease remains clinically inactive. In contrast, a person who inherits two sickle cell genes has sickle cell disease. Early childhood diagnosis and effective treatments have substantially improved the prognosis for individuals with the disease.


Sickled cells carry normal amounts of hemoglobin; however, the cells tend to clump together because of their distinct shape. Clumping increases blood viscosity and can result in capillary obstruction. This is known as a sickle cell crisis (vaso-occlusive crisis). Decreased circulation, edema formation, tissue ischemia, and severe pain ensue. Sickle cell crisis occurs more frequently at night and is associated with a number of precipitants, including:



• Cold ambient temperature


• High altitude


• Infection


• Metabolic or respiratory acidosis


• Stress


If the ischemic state is not corrected, local tissue necrosis soon follows, which can lead to organ dysfunction.


Children generally do not show symptoms of sickle cell anemia until after 4 months of age. Infants and young children may experience hand-foot syndrome (edema secondary to venous stasis). The incidence of ischemic stroke is increasing in children with sickle cell anemia. The disease has a significant psychological influence on patients because of altered body image, frequently missed days of school or work, sexual problems, depression, and fear of a shortened lifespan.



Signs and Symptoms




• Acute, severe pain


• Acute pain episodes are the single biggest reason for ED visits and hospitalizations in adults with sickle cell disease.2

• Pain described as throbbing, achy, sharp, or dull with sudden onset.3

• Weakness due to chronic anemia


• Pallor


• Jaundice resulting from rapid breakdown of RBCs


• Frequent infections resulting from splenic damage



Complications




• Acute chest syndrome


• Defined as a new pulmonary infiltrate on chest radiograph, fever, cough, worsening anemia, and chest pain

• Leading cause of morbidity and mortality and a predictor of early death in sickle cell disease3

• Chronic hemolytic anemia, transient aplastic crisis


• Frequent infections such as pneumonia, meningitis, and osteomyelitis


• Cholelithiasis and cholecystitis


• Delayed sexual maturation or priapism


• High incidence of spontaneous abortion, perinatal mortality, and maternal mortality


• Renal failure


• Bone disease, particularly infarction leading to avascular necrosis of the femoral head


• High-output cardiac failure


• Autosplenectomy—almost complete disappearance of the spleen because of progressive fibrosis and shrinkage


• Pulmonary embolus


• Cor pulmonale resulting from pulmonary hypertension, a common occurrence in adults


• Chronic skin ulcers


• Jaundice, hepatomegaly, and hepatic infarction


• Blindness resulting from blockage of retinal blood vessels



Diagnostic Procedures




• Elevated reticulocyte count


• Sickled cells on smear


• Leukocytosis


• Thrombocytosis


• Decreased hemoglobin and hematocrit



Therapeutic Interventions




• Pain management is a priority.


• Many patients report their sickle cell pain management has been inadequate and suboptimal.2

• For mild to moderate pain, use medications such as NSAIDs, acetaminophen (Tylenol), and tramadol (Ultram).

• For moderately severe to severe pain, use medications such as opioids (codeine, hydrocodone, oxycodone) and ketorolac (Toradol).

• Supplemental oxygen.


• Intravenous rehydration.


• Local application of heat to areas of pain.


• Patients may require blood transfusion if they are significantly anemic.


• Antibiotics if infection is present.


• Maintain warm environment.


• Provide emotional support.



Hemophilia


Hemophilia is a congenital coagulation disorder caused by lack of one of two essential circulating plasma proteins: factor VIII and factor IX. It is an inherited sex-linked (passed from mother to son) disease found primarily in males.


Hemophilia produced by a factor VIII deficiency is referred to as hemophilia A, or classic hemophilia. A majority of patients with hemophilia A produce factor VIII, but it is either entirely nonfunctional or operates below normal capacity. A factor IX deficiency is called hemophilia B, or Christmas disease. Type C, rarely seen in the U.S. population, results from a factor XI deficiency.


Normal clotting is an intricate process that depends on a number of specifically sequenced events:



• Factors VIII, IX, and XI are proteins normally found in the plasma.


• Clotting factors circulate in an inactive form until the clotting cascade, stimulated by a cut or bruise, initiates factor activation.


• Factors in this cascade are activated in a domino-like fashion until fibrinogen becomes fibrin and a clot is formed.


• If any one factor is inactivated or removed from the sequence, proper clot formation will be impeded.


In hemophilia the normal physiological events required for formation of a clot are disrupted by the ineffectiveness of factor VIII, IX, or XI:



• Hemophilia prevents the formation of a firm, fibrin clot; instead, patients with hemophilia have soft, unstable clots. The result is not faster bleeding but rather continuous bleeding.4


• Hemophilia severity depends on the extent of factor function in each individual patient.


• Hemophilia is typically diagnosed in infancy but some mild cases go undetected until childhood or adolescence.

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Aug 9, 2016 | Posted by in EMERGENCY MEDICINE | Comments Off on Hematologic and Immunologic Emergencies

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