Hem/Onc Flashcards
For a patient presenting for follow-up of monoclonal gammopathy of undetermined significance, which one of the following findings would be most concerning for progression to multiple myeloma? (check one)
A serum albumin level that is more than 1 g/dL below the lower limit of normal
A serum calcium level that is more than 1 mg/dL above the upper limit of normal
A hemoglobin level that is 2 g/dL above the upper limit of normalThe presence of 3 or more RBCs/hpf on microscopic urinalysis
The presence of 3 or more RBCs/hpf on microscopic urinalysis
An osteoblastic lesion seen on a skeletal radiograph
A serum calcium level that is more than 1 mg/dL above the upper limit of normal
Multiple myeloma (MM), a malignancy of plasma cells, represents 1.6% of all cancer cases and approximately 10% of the hematologic malignancies seen in the United States. Patients with monoclonal gammopathy of undetermined significance (MGUS) have a 1% annual risk of progression to MM. Patients who have progressed to MM typically manifest one or more of the classic CRAB findings: calcium (hypercalcemia of >11 mg/dL), renal impairment (a creatinine level >2 mg/dL or an estimated glomerular filtration rate <40 mL/min/1.73 m2), anemia (a hemoglobin level <10 g/dL), and bone involvement (osteolytic lesions, pathologic fractures, and/or severe osteopenia), which represent evidence of end-organ disease. Of the options listed, only hypercalcemia raises concern for progression of MGUS to MM. While patients with MM often have an elevated total serum protein level, the increase is from plasma cell–related proliferation and the resulting monoclonal protein production, not from an increase in albumin. Patients with MM would be expected to have a decrease in the hemoglobin level, not an increase. Renal manifestations typically involve a decrease in the serum creatinine level rather than microscopic hematuria. Finally, bone involvement in MM includes lytic, as opposed to blastic, lesions.
A 30-year-old female sees you because of increasing fatigue. She has no chronic medical problems and reports no recent acute illnesses. She recalls being told that she was mildly anemic after the birth of her daughter 3 years ago. The anemia resolved after 3 months of oral iron supplementation. The patient’s menstrual periods are regular and last approximately 6 days, with heavy bleeding for the first 3 days then moderate to mild flow for approximately 3 days. She denies epistaxis, black stools, or other signs of bleeding.
On examination her temperature is 36.7°C (98.1°F), pulse rate 93 beats/min, respiratory rate 16/min, and blood pressure 116/58 mm Hg. The remainder of her physical examination is unremarkable. A CBC is notable for a hemoglobin level of 10.9 g/dL (N 12.0–16.0) and a mean corpuscular volume of 70 !m3 (N 78–102).
Which one of the following serum levels would be most appropriate for further evaluating her microcytic anemia at this point? (check one)
Ferritin
Folate
Erythropoietin
Hemoglobin A1c
TSH
Ferritin
After confirmation of anemia and microcytosis on a CBC, a serum ferritin level is recommended (SOR C). If the ferritin level is consistent with iron deficiency anemia, identifying the underlying cause of the anemia is the priority. A common cause of iron deficiency anemia in premenopausal adult women is menstrual blood loss. If the serum ferritin level is not consistent with iron deficiency anemia, the next stage of the evaluation should include a serum iron level, total iron-binding capacity (TIBC), and transferrin saturation (SOR C). Iron deficiency anemia is still probable if the serum iron level and transferrin saturation are decreased and TIBC is increased. It is more likely anemia of chronic disease if the serum iron level is decreased and the TIBC and transferrin saturation are decreased or normal. Other laboratory tests that may help in differentiating the cause of microcytosis include hemoglobin electrophoresis, a reticulocyte count, and peripheral blood smears.
In an adult who has a critical illness but no history of cardiac disease, the threshold for transfusion of red blood cells should be a hemoglobin level of ?
(check one)
6 g/dL
7 g/dL
8 g/dL
9 g/dL
10 g/dL
7 g/dL
The threshold for transfusion of red blood cells should be a hemoglobin level of 7 g/dL in adults and most children.
A 78-year-old asymptomatic male is found to have a platelet count of 90,000/mm3 (N 150,000–300,000) and a slightly decreased WBC count. Which one of the following would be most consistent with a diagnosis of myelodysplastic syndrome?
(check one)
A normal RBC count and indices
Normocytic anemia
Microcytic anemia
Macrocytic anemia
Polycythemia
Macrocytic anemia
Myelodysplastic syndrome is a hematologic malignancy with a predisposition to leukemic transformation. It can present with findings of anemia, thrombocytopenia, neutropenia, or any combination of these. Anemia occurs in 80%–85% of patients and is typically macrocytic.
Which one of the following is a classic finding in multiple myeloma?
(check one)
Hypokalemia
Bone pain
Polycythemia
Hepatic failure
Insomnia
Bone pain
Multiple myeloma can be asymptomatic, but it becomes symptomatic when there is organ damage or other abnormalities, including renal insufficiency, elevated calcium, anemia, and bone disease.
The majority of patients have bone pain, but hypocalcemia is not common. Hypokalemia almost never occurs, and both hepatic failure and insomnia are not usual signs of multiple myeloma. Anemia typically occurs either because of renal failure or infiltration of the bone marrow by myeloma cells. Polycythemia does not occur.
For most patients, which one of the following is the most effective treatment for anemia of chronic disease?
(check one)
Elemental iron
Erythropoietin
Prednisone
Optimal management of the underlying disorder
Combined therapy with oral iron, vitamin B12, folic acid, and erythropoietin 0.3%/dexamethasone 0.1% (Ciprodex) topically
Optimal management of the underlying disorder
There is no specific therapy for anemia of chronic disease except to manage or treat the underlying disorder. Iron therapy is of no benefit, but erythropoietin may be helpful in some patients. There is no available data to suggest that combination therapy or prednisone is beneficial for this disorder.
Activated protein C resistance (factor V Leiden) is most commonly found in patients with?
(check one)
Hemolytic anemia
Carcinoma of the lung
Familial hypercholesterolemia
Venous thrombotic disease
Cystic fibrosis
Venous thrombotic disease
Venous thrombosis, both acute and recurrent, is associated with several hematologic abnormalities, in addition to the well-known factors of trauma, surgery, malignancy, sepsis, and oral contraceptive use. Notably, activated protein C resistance (factor V Leiden) has been found to be one of the most common hereditary causes of thrombophilia.
An otherwise healthy 1-year-old white male has a screening hemoglobin level of 10.5 g/dL (N 11.3–14.1), a mean corpuscular volume of 68 fL (N 71–84), and an undetectable serum lead level. What should be your next step? (check one)
A therapeutic trial of iron for 1 month
A serum ferritin level
An erythrocyte protoporphyrin level
Hemoglobin electrophoresis
Bone marrow examination
A therapeutic trial of iron for 1 month
It is important to screen for anemia during late infancy. Iron deficiency is the most common cause of anemia in this age group. There is evidence that persistent iron deficiency in childhood may have a negative impact on cognitive development. A therapeutic trial of iron is the best approach to the treatment of iron deficiency in late infancy. If the anemia fails to respond, investigating other causes of anemia is indicated.
An obese 37-year-old white female sees you because of fatigue. She is otherwise asymptomatic and has normal vital signs. A complete physical examination is unremarkable with the exception of obesity. A CBC shows no anemia, but her WBC count is 12,500/mm3 (N 4500–11,000). A TSH level and a comprehensive metabolic panel are normal. She does not take any medications and has not had any recent illnesses.
Which one of the following would be most appropriate at this point? (check one)
Reassurance that her leukocytosis is likely caused by her obesity and counseling about weight loss
A repeat CBC with differential and a review of the peripheral smear
A blood culture
Flow cytometric testing
Referral to a hematologist for further workup
A repeat CBC with differential and a review of the peripheral smear
Leukocytosis is a relatively common finding with many possible etiologies. For most cases without a clear cause, a repeat CBC with differential and a peripheral smear review are indicated to confirm leukocytosis, determine subtypes, and look for concerning abnormalities on the smear. Given this patient’s fatigue, a hematologic referral may be indicated if leukocytosis is confirmed on repeat testing. Similar recommendations would apply to flow cytometry testing. Blood cultures are not necessary in cases of leukocytosis without evidence of infection. Obesity can cause leukocytosis, but because of the patient’s fatigue it would not be appropriate at this time to attribute the leukocytosis to obesity alone.
A 25-year-old white female with heavy menstrual periods is noted to have a hemoglobin level of 9.8 g/dL (N 12.0–16.0). The red cell distribution width is 16.0% (N 11.5–14.5) and the mean corpuscular volume is 75 µm3 (N 78–102). The appropriate treatment for this condition can be enhanced by the use of: (check one)
Antacids
Soy milk
Iced tea
Bran
Ascorbic acid
Ascorbic acid
This patient has iron deficiency anemia. There are several substances that decrease the absorption of iron, including antacids, soy protein, calcium, tannin (which is in tea), and phytate (which is found in bran). Since an acidic environment increases iron absorption, ascorbic acid (vitamin C) can enhance absorption of an iron supplement.
A 45-year-old female with rheumatoid arthritis has a hemoglobin level of 9.5 g/dL (N 11.5-16.0). Her arthritis is well controlled with methotrexate. Further evaluation reveals the following:
Hematocrit…………29.0% (N 35.0-47.0)
Mean corpuscular volume…………78 µm3 (N 80-98)
Platelets…………230,000/mm3 (N 150,000-400,000)
WBCs…………6900/mm3 (N 4000-11,000)
Differential…………normal
Serum iron…………15 µg/dL (N 50-170)
Total iron binding capacity…………150 µg/dL (N 45-70)
Iron saturation…………10% (N 15-50)
Serum ferritin…………7 ng/mL (N 12-150)
Reticulocyte count…………8 x 109/L (N 10-100)
Stool guaiac…………negative x 3
Which one of the following would be the most appropriate next step?
(check one)
Evaluation for a source of blood loss
Hemoglobin electrophoresis to screen for thalassemia
Stopping the methotrexate and beginning an alternative treatment for rheumatoid arthritis
No further evaluation
Evaluation for a source of blood loss
Anemia of chronic disease is characterized by the underproduction of red cells, due to low serum iron caused by the uptake of iron by the reticuloendothelial system. Total-body iron stores are increased but the iron in storage is not available for red cell production. This anemia is normochromic and normocytic, and is associated with a reduction in iron, transferrin, and transferrin saturation. Ferritin is either normal or increased, reflecting both the increased iron within the reticuloendothelial system and increases due to immune activation (acute phase reactant). In iron deficiency anemia, total-body iron levels are low, leading to hypochromia and microcytosis, low iron levels, increased transferrin levels, and reduced ferritin levels. This patient’s anemia is most likely multifactorial, with anemia of chronic disease and drug effects playing a role. However, she also has iron deficiency, and searching for a source of blood loss would be important. With thalassemia, marked microcytosis is seen, and with hemolysis, slight macrocytosis and an increased reticulocyte count would be expected.
A 60-year-old male has moderate anemia, with a suggestion of hemolysis on a peripheral blood smear. Which one of the following patterns would be consistent with the presence of hemolysis? (check one)
Elevated LDH, decreased haptoglobin, elevated indirect bilirubin
Elevated LDH, elevated haptoglobin, decreased indirect bilirubin
Decreased LDH, elevated haptoglobin, elevated indirect bilirubin
Decreased LDH, decreased haptoglobin, elevated indirect bilirubin
Decreased LDH, decreased haptoglobin, decreased indirect bilirubin
Elevated LDH, decreased haptoglobin, elevated indirect bilirubin
Hemolytic anemia is established by reticulocytosis, increased unconjugated bilirubin, elevated lactate dehydrogenase (LDH), decreased haptoglobin, and peripheral blood smear findings.
A 34-year-old female with menorrhagia is found to have iron deficiency anemia. Which one of the following is true regarding the treatment of this problem with oral iron? (check one)
An acidic environment enhances the absorption of iron from the gastrointestinal tract
Iron is absorbed better if taken with food
Diarrhea is a common complication
Iron supplementation can be discontinued once the hemoglobin reaches a normal level
Sustained-release formulations increase the total amount of iron available for absorption
An acidic environment enhances the absorption of iron from the gastrointestinal tract
Oral iron is absorbed better with an acidic gastric environment, which can be accomplished with the concomitant administration of vitamin C. Agents that raise gastric pH, such as antacids, proton pump inhibitors, and H2 blockers, should be avoided if possible. Oral iron absorption is improved if the iron is taken on an empty stomach, but this may not be well tolerated because gastric irritation is a frequent side effect. Constipation also is common with oral iron therapy. Iron therapy should be continued for several months after the hemoglobin reaches a normal level, in order to fully replenish iron stores. Sustained-release oral iron products provide a decreased amount of iron for absorption.
You see a 45-year-old male with fatigue, arthralgias, and mildly elevated liver function tests. You are considering hereditary hemochromatosis as a possible diagnosis.
Which one of the following should you order first? (check one)
A serum ferritin level and transferrin saturation
Genetic testing for HFE mutations
T2-weighted MRI for hepatic iron concentration
A liver biopsy
A serum ferritin level and transferrin saturation
The initial tests used in the workup for suspected hemochromatosis are a serum ferritin level and transferrin saturation. A transferrin saturation >45% and a serum ferritin level >300 ng/mL in men or >200 ng/mL in women are indicative of iron overload and highly suggestive of hereditary hemochromatosis. A serum iron level is ordered as part of transferrin saturation testing, but an elevated iron level by itself is not as sensitive or specific as the other tests. Other etiologies of iron overload should be ruled out, including liver disease, alcohol abuse, and metabolic syndrome. If no secondary etiologies are found, genetic testing would be appropriate to identify HFE mutations indicating hereditary hemochromatosis. Genetic testing should not be performed in a patient without iron overload or a family history of hereditary hemochromatosis. MRI may help determine the risk of developing cirrhosis, and a liver biopsy is used to determine the amount of liver damage.
You see a 5-year-old female for the first time for a new patient visit. Her mother notes that she tires easily and sometimes cannot keep up with other children her age. Laboratory studies reveal the following:
WBCs………………………………………………………6500/mm3 (N 5000–14,500)
RBCs……………………………………………………….5.6 million/mm3 (N 3.90–5.30)
Hemoglobin…………………………………………….9.1 g/dL (N 11.5–15.5)
Hematocrit……………………………………………..27% (N 34–40)
Platelets…………………………………………………..220,000/mm3 (N 150,000–450,000)
Mean corpuscular volume……………………68 μm3 (N 75–87)
Mean corpuscular hemoglobin……………28 pg/cell (N 24–30)
Mean corpuscular hemoglobin
concentration………………………………….34 g/dL (N 32–36)
Red cell distribution width…………………….11% (N 11.5–15.0)
Ferritin………………………………………………………150 ng/mL (N 7–140)
Transferrin saturation………………………….40% (N 15–50)
A peripheral smear shows target cells, microcytic cells, red cell fragments, teardrop cells, and nucleated RBCs.
Which one of the following is the most likely etiology of this patient’s anemia? (check one)
Aplastic anemia
Iron deficiency
Megaloblastic anemia
Myelofibrosis
Thalassemia minor
Thalassemia minor
This patient most likely has thalassemia minor and will need further genetic testing to confirm the diagnosis. Thalassemia minor is associated with microcytic anemia. Thalassemia can be differentiated from iron deficiency based on low red cell distribution width (RDW), elevated reticulocyte count, normal or slightly elevated RBC count, slightly elevated ferritin, and nucleated RBCs on peripheral smear. Aplastic anemia is associated with a poor reticulocyte response and low counts. Iron deficiency is associated with an elevated RDW; a low reticulocyte count; and low RBC, ferritin, and transferrin saturation levels. Megaloblastic anemia is typically associated with an elevated mean corpuscular volume. Myelofibrosis is associated with bone marrow failure and pancytopenia.
