Hematology/Oncology Flashcards
If a patient presents with mucosal or petechial hemorrhages, what is the most likely bleeding disorder?
“Generally, petechiae and mucosal bleeding are the result of platelet problems (e.g., mild von Willebrand disease and thrombocytopenia) while hemarthrosis and hematomas are the result of a factor deficiency. Severe von Willebrand disease may present with hemarthrosis and hematomas”
When are most hemophiliac patients identified?
“Up to half of hemophiliac patients do not bleed after circumcision. Depending on the severity of factor deficiency, the diagnosis may not be made until the child is very active or even in adulthood, after surgery, etc”
Define hemophilia A.
“Hemophilia A is an X-lined deficiency of factor VIII, which presents with hematomas, bleeding, and hemarthrosis (and not generally mucosal bleeding or petechiae”
Define hemophilia B.
“Deficiency of factor IX, or hemophilia B, also known as “Christmas Disease,” is also X-linked but is much less common”
Hemophilia most often occurs in females. T or F
“Hemophilia rarely occurs in females but can occur in two situations: (1) the female patient is a heterozygote who has early inactivation of the second X chromosome during embryogenesis or (2) if both parents are carriers—in which case the father would have the disease overtly”
Name 2 blood tests indicated for patients with suspected bleeding disorders?
CBC, PT, PTT, PFA-100, platelet count. The PTT is actually the most sensitive test for hemophilia. The PFA-100 (Platelet Function Assay) tests for (appropriately enough) platelet functioning and will be abnormal in von Willebrand disease”
For a patient presenting with a known bleeding disorder and a hemarthrosis, what activity level is recommended?
“For a hemarthrosis or other “minor” bleeding, you should maintain a factor level of 30–40% for 72 hours. For more serious bleeding (intracranial, for example), maintain a level of 80–100% for 10 days”
For a patient with a mild exacerbation of their bleeding disorder, name 2 treatments.
FFP, cryoprecipitate, factor, “Patients with mild hemophilia (and some types of von Willebrand disease) will respond to desmopressin with a transient increase in serum von Willebrand factor (vWF) and factor VIII”
What is ITP?
isolated low platelet count (thrombocytopenia) with normal bone marrow and the absence of other causes of thrombocytopenia. It causes a characteristic purpuric rash and an increased tendency to bleed. Two distinct clinical syndromes manifest as an acute condition in children and a chronic condition in adults. The acute form often follows an infection and has a spontaneous resolution within 2 months. Chronic idiopathic thrombocytopenic purpura persists longer than 6 months without a specific cause.
What causes the acute form of ITP?
“Both infections and the MMR vaccine have been linked to ITP”
Name two interventions that will result in a sustained increase in platelet count.
IVIG, Steroids, Spleenectomy. Transfused platelets will just be chomped up by Mr. Spleen. However, platelets can be used to temporize if a patient must go to the OR, etc. Most children can simply be observed. The only indication for treatment is bleeding. All of the treatments have a downside. Splenectomy is associated with a risk of sepsis. If possible, delay splenectomy until after the child is over 5 years old. Steroids may cause behavioral problems and long-term problems such as avascular necrosis. IVIG leads to a temporary increase in platelets that may last several weeks but can be associated with renal injury and anaphylaxis among other adverse effect”
What is the treatment for ITP in adults and children?
In children it is mostly supportive. “In adults, Treatment incudes (in order) steroids, Rho(D) immunoglobulin for Rh-positive patients, IVIG (which causes a transient rise in platelet numbers), and splenectomy. Other treatment options for refractory cases include rituximab or various immunosuppressive agents. The thrombopoietin-receptor agonists, romiplostim and eltrombopag, stimulate platelet production and can also be used in ITP.”
What is gestational thrombocytopenia?
“This patient likely has gestational thrombocytopenia, a condition that occurs in up to 5% of pregnant women. It is characterized by mild thrombocytopenia occurring in late gestation; the platelet count is usually >70,000/mm3 (two-thirds are between 130,000/mm3 and 150,000/mm3). The condition resolves after delivery and is not associated with severe neonatal thrombocytopenia. No specific change in routine obstetrical care is warranted, although the anesthesiologist placing an epidural may want a follow-up platelet count closer to the time of delivery”
Name two causes of both a prolonged PT and PTT.
Severe liver disease, DIC & factor 2, 5 & 10. The three factor deficiencies that may prolong both PT and PTT are II, V, and X. Both PTT and PT may be prolonged due to severe liver disease and DIC as well. Mild vitamin K deficiency or mild liver disease generally affects the PT only. Generally, heparin affects PTT, and warfarin affects PT.”
What are the general indications for transfusion?
“Hemodynamic instability due to bleeding unresponsive to 2 L of saline, Preoperative Hb of 7–8 gm/dL, Elderly patient after a myocardial infarction (MI) with a hematocrit <7 gm/dL in postoperative patients who are hemodynamically stable.”
What are the signs of a transfusion reaction and how do you treat it?
“hemolytic transfusion reaction, which is generally the result of an ABO incompatibility. Patients may exhibit nausea, flushing, dyspnea, oliguria, back pain, and hypotension. Other findings include markers of hemolysis: hemoglobinuria, elevated serum-free Hb, reduced haptoglobin, and elevated bilirubin. Patients are positive for direct antiglobulin (Coombs) test. Therapy includes IV saline at a high enough rate to initiate a brisk diuresis and prevent Hb from precipitating in the kidneys causing acute tubular necrosis”
What is the most effective way to emergently lower the PTT in a patient taking warfarin?
“FFP contains all the soluble plasma proteins found in whole blood, including the vitamin K-dependent factors that are depleted by warfarin. If more sustained reversal is desired, the simultaneous administration of vitamin K is effective. The preferred route of administration of vitamin K is oral. Giving vitamin K IV is second best—it is associated with a risk of anaphylaxis and lowers INR the same degree as oral vitamin K at 24 hours. Avoid vitamin K SQ or IM, which are less effective than PO and IV routes”
What is neutropenic fever and how is it treated?
development of fever, often with other signs of infection, in a patient with neutropenia. It is most generally recognized as a complication of chemotherapy when it is myelosuppressive (suppresses the bone marrow from producing blood cells).
Generally, patients with febrile neutropenia are treated with empirical antibiotics covering broad spectrum gram negative and positive organisms until the neutrophil count has recovered (absolute neutrophil counts greater than 500/mm3) and the fever has abated; if the neutrophil count does not improve, treatment may need to continue for two weeks or occasionally more. In cases of recurrent or persistent fever, an antifungal agent should be added.
A patient with a history of small cell lung cancer presents with thoracolumbar pain, what should your next step be?
“Any patient with active malignancy complaining of back pain should be investigated for metastasis. While a plain film of the spine may be useful, the gold standard is MRI”
The patient with the spinal cord mass, as result of a probable metastasis, what should your next step be?
“Patients with spinal cord compression who have aggressive interventions are more likely to retain function, including ambulation and bowel and bladder control. Surgical decompression is an option. Steroids will help reduce edema surrounding the tumor and hopefully will relieve pressure on the cord. Radiation can often provide symptomatic relief and reduce the likelihood that the tumor will spread locally to impinge on the cord. Hospice is always an option.
A female patient who is 32 years old with a distant history of Hodkins disease treated with chemotherapy and radiation to the chest presents with complaints of shortness of breath and chest pain. Her physical exam is unremarkable and CBC is normal. What should you consider?
“The point here is that patients with a history of Hodgkin disease and chest radiation are at risk for a wide range of complications—even years after the disease has been successfully treated. Even though 80% of Hodgkin patients have long-term disease-free survival, one in six patients can be expected to die from late effects of therapy. Although CAD would be extremely unusual in a normal 32-year-old, a patient with a history of chest radiation has a relative risk of coronary disease of 5–10 times that of age-matched controls. thyroid disease, is highly likely, with over 50% of patients treated with chest radiation requiring thyroid hormone replacement. A TSH would be adequate screening. There is a high risk for secondary malignancy, including breast cancer, lung cancer, leukemia, sarcoma, and non-Hodgkin lymphoma (NHL). Finally, you should always be concerned about recurrence”
Name two long-term concerns for a patient who is treated successfully in her early 20s for Hodgkinson disease with chemotherapy and chest radiation.
“Female patients who were treated for Hodgkin disease with chemotherapy and radiation prior to age 20 have up to a 35% incidence of breast cancer by age 40. The typical latency period is 15 years. National guidelines recommend that annual mammography of Hodgkin survivors treated with chest irradiation should begin 5–8 years post-treatment, or age 40, which ever comes first. Smokers with a history of Hodgkin disease have a 20-fold increased chance of developing lung cancer when compared to nonsmokers with a history of Hodgkin disease. Also, female patients with Hodgkin disease have a 69% incidence of premature ovarian failure if treated for their cancer before age 29 and up to 96% if treated after age 30. While these statistics are improving with newer chemotherapy regimens, a patient should seek early referral to a fertility specialist if she desires pregnancy but is unable to conceive”
What are the characteristics of tumor lysis syndrome?
“tumor lysis syndrome, which can occur in a patient with a highly responsive leukemia or a bulky lymphoma being treated with chemotherapy (it rarely occurs without treatment). Tumor lysis syndrome occurs when there is rapid release of intracellular contents into the bloodstream. It is characterized by high potassium, high phosphorus, high uric acid, and low calcium. Patients may experience renal failure, arrhythmias, fatigue, muscle cramps, and tetany”
What’s the most likely cause of renal failure in a patient with tumor lysis syndrome?
“Patients with tumor lysis syndrome have renal failure secondary to uric acid nephropathy. This is caused by the precipitation of uric acid in the kidney, and it can be prevented by the use of allopurinol prior to the administration of chemotherapy.”
With a patient with renal failure from tumor lysis syndrome, how should you treat the renal failure?
“Allopurinol and oral calcium will not help this situation. The patient already has renal failure from his tumor lysis syndrome. While he may benefit from preventive measures, including aggressive hydration and allopurinol prior to undergoing chemotherapy, none of these measures are going to help his current renal failure.”
What is the treatment for hyperkalemia?
“Treatment may include IV calcium gluconate, insulin and dextrose, and oral sodium polystyrene sulfonate (Kayexalate). Note that sodium bicarbonate for hyperkalemia has fallen out of favor.
What are the two questions one needs to answer in order to determine the cause of it elevated hematocrit?
“1) Is it due to increased RBC mass or decreased plasma volume? (2) Is it primary erythrocytosis or secondary?”
What are the potential causes of secondary elevated hematocrit level?
“alcoholic cirrhosis can lead to hepatocellular carcinoma which, along with other malignancies, can result in overproduction of erythropoietin, causing an elevated hematocrit. Diuretics decrease plasma volume, causing an apparent elevation of hematocrit. Testosterone injections may cause polycythemia. Finally, he has a significant smoking history that may produce a secondary polycythemia due to hypoxia and cor pulmonale.”
What is essential thrombocytopenia and what causes it?
“most common myeloproliferative disorder in the United States. ET is more common in females. Although patients are typically older when diagnosed, it is not uncommon in younger patients. Patients with ET have a higher rate of mortality than matched controls due to risk of thrombosis (arterial > venous) and bleeding events. In order to diagnose ET, other causes of thrombocytosis (e.g., inflammation, iron deficiency, recent surgery, infection, bleeding, and malignancy) must be excluded. A bone marrow biopsy may be helpful in establishing the diagnosis by demonstrating adequate iron stores and ruling out chronic myelogenous leukemia (CML) or myelodysplasia”
What is the most common cause of an abnormally high platelet count?
“The most common cause of an abnormally high platelet count is reactive thrombocytosis, which can result from iron deficiency, infection, inflammation, or malignancy. There is no increase in bleeding or clotting risk in patients with reactive thrombocytosis”
What’s the first indicator that iron replacement is working in iron deficient anemia?
Reticulocyte count
Ferritin is the most useful test in iron deficient anemia. T or F
False. “Ferritin is not a useful test for iron deficiency in hospitalized patients or in those who are chronically ill. Ferritin is an acute-phase reactant and thus may be elevated in these patients even when the patient has iron-deficiency anemia (where the ferritin should be low).”
Name two causes of failure for iron replacement therapy in iron deficient anemia.
“Anything that neutralizes the stomach pH will interfere with absorption including PPIs, antacids, and loss of acid producing cells (e.g., pernicious anemia). Other GI diseases (celiac disease, H. pylori) can also interfere with iron absorption. Tea and some green leafy vegetables can also reduce iron absorption.”
Name two ways to increase iron absorption.
“Vitamin C (supplements or orange juice) enhances iron absorption and should be considered if a patient is not responding to iron therapy. Meat can also increase iron absorption (which we hate to say because one of us is a vegetarian … however, the truth hurts).”
If oral iron is not tolerated, what other options for replacement are available?
“If oral iron preparations are not tolerated, IV iron preparations are available. Intramuscular preparations are best avoided due to pain at the injection site, skin discoloration, and risk for infection. Options for IV replacement include iron dextran and iron sucrose”
What are the side effects of the iron replacement IVs and, more importantly, which one has less side effects associated?
“Iron dextran carries a risk of anaphylaxis in 0.6–2.3% of patients and other side effects in up to 25% of patients, including bronchospasm, flushing, headache, fever, urticaria, nausea, vomiting, hypotension, seizures, myalgia, arthralgia, and increased thromboembolic events. Iron sucrose has a lower incidence of side effects—typically nausea, constipation, diarrhea, or a transient minty taste—and may be given to patients who have had a previous reaction to iron dextran. “A” is incorrect because calcium will interfere with iron absorption. Not only that but also she has already said she would not take additional oral iron”
How does anemia of chronic disease present?
“Anemia of chronic disease (previously known as anemia of inflammation) is a hypoproliferative anemia that occurs in the setting of chronic infection, inflammation, malignancy, heart failure, diabetes, and other serious health conditions. The anemia is usually mild and characterized by low serum iron, increased ferritin (remember that ferritin is an acute-phase reactant and these patients often have inflammation), decreased serum transferrin, normal (or low) serum soluble transferrin receptor level, and decreased transferrin saturation (see below for more on the soluble transferrin receptor). In addition, the reticulocyte count is typically low, the erythropoietin may be mildly elevated, and the peripheral smear may show hypochromic, microcytic RBCs or normochromic, normocytic RBCs”
With iron levels being screwed up in both, what is the gold standard of diagnosis between iron deficient anemia and anemia if chronic disease?
“If differentiation between iron deficiency anemia and anemia of chronic disease is not apparent, a bone marrow biopsy can be obtained to assess iron stores.”
How does a child present with lead poisoning?
“Lead poisoning should be considered in a child presenting with symptoms of encephalopathy and anemia with basophilic stippling on RBCs. Basophilic stippling occurs when ribosome precipitates litter the RBCs and can be seen in alcohol abuse, thalassemias, and heavy metal poisoning”
What diseases are associated with basophilic stippling?
Basophilic stippling occurs when ribosome precipitates litter the RBCs and can be seen in alcohol abuse, thalassemias, and heavy metal poisoning”
