PAEasy Hem Onc Flashcards
A patient who is an Ashkenazi Jew and is homozygous for Factor XI deficiency requires abdominal surgery. Which hemostatic laboratory testing outcomes would you expect to find?
A. Decreased platelets, normal aPTT, prolonged PT
B. Decreased platelets, prolonged aPTT, normal PT
C. Normal platelets, normal aPTT, prolonged PT
D. Normal platelets, prolonged aPTT, normal PT
E. Normal platelets, prolonged aPTT, prolonged PT
The answer is D.
EXPLANATION: Patients with factor XI deficiency have a prolonged aPTT, normal PT, and normal platelets count.
In a person with normal marrow function what is the mean life span of platelets?
A. 3 to 6 days
B. 7 to 10 days
C. 11 to 14 days
D. 15 to 18 days
E. 19 to 22 days
The answer is B.
A 67-year-old male presents for his annual physical in February. On examination, you note nontender cervical and axillary adenopathy. He states that he has felt those lumps for several weeks, has noted feeling more fatigued, and has decided that he had a cold. On further inspection, you note a mildly enlarged spleen, and the patient states that he hasn’t been eating as much as he usually does. A CBC reveals an elevated lymphocyte count. These findings are most consistent with which diagnosis?
A. Acute infection lymphocytosis
B. Chronic lymphocytic leukemia
C. Rickettsiosis
D. Stress lymphocytosis
E. Systemic lupus erythematosus
The answer is B.
EXPLANATION: The findings in this patient are consistent with chronic lymphocytic leukemia. Rickettsiosis and systemic lupus erythematosus tend to cause a lymphocytopenia rather than lymphocytosis. Acute infection lymphocytosis do not have either lymph node enlargement or splenomegaly, and stress lymphocytosis is short lived following trauma, surgery, or other insults to the body.
A 3-year-old white male with low grade fever, lethargy, and fatigue for several weeks is seen on repeat visit. His mother states that nothing has helped. He has been trialed on a course of antibiotics without improvement. He has petechiae to his lower extremities and pallor throughout. What other physical finding is likely to be found in this patient?
A. Bone pain
B. Cranial nerve palsy
C. Epidural spinal cord compression
D. Painless enlargement of the scrotum
E. Subcutaneous nodules
The answer is A.
EXPLANATION: All physical findings are possible in this patient, who has acute lymphoblastic leukemia (ALL). However, of those listed, bone pain is the most common. All other choices are extremely rare findings (usually less than 1%). In children presenting with ALL, the frequency of bone pain or limp can be more than 25% of cases.
A 29-year-old female complains of a two-month history of easy bruising. She describes that the bruising is located primarily on her shins, but has noted them on other areas as well. She also describes red freckles on her lower extremities. On exam, you note non-blanching and non-palpable purpura to both legs and petechiae. She denies recent illnesses and states that she has essentially been feeling fine. The PE is normal other than the skin findings. Which diagnostic study would be most helpful as a first choice?
A. Antiplatelet antibody test
B. Complete blood count
C. Direct antiglobulin test
D. Reticulocyte count
E. Thyroid function
The answer is B.
EXPLANATION: This patient is exhibiting signs of Idiopathic (Immune) thrombocytopenic purpura. The diagnosis is based on history, physical examination, blood count, and blood film. The American Society of Hematology guidelines recommend no further diagnostic studies. The other studies may be useful if you believe another underlying disease is causing the symptoms and thrombocytopenia, but a complete blood count should be sufficient.
A 24-year-old male with thalassemia major who has received adequate transfusions, chelation therapy, and regular health checkups is in to establish care. He is 5’4” with a BMI of 17. He eats a balanced healthy diet and gets regular exercise. You know that there are complications of this disease. What is the best next step for this patient?
A. Increase threshold for blood transfusions
B. Maintain sustained reduction of body iron
C. Obtain a Dexa scan for osteoporosis
D. Obtain an ECHO to evaluate for cardiac siderosis
E. Obtain regular testosterone levels and treat
. The answer is B.
Maintaining sustained reductions in body iron has demonstrated increased overall survival rates through reductions in cardiac disease specifically due to siderosis. While these patients are at increased risk for osteoporosis and cardiac siderosis, the next best step in this patient is to maintain reduced iron levels. There is no place for increased blood transfusion or obtaining regular testosterone levels.
A 27-year-old female carries a diagnosis of hereditary spherocytosis. She has been noted to have a few small gallstones that currently do not require a cholecystectomy. While her mean corpuscular hemoglobin concentration is increased, she maintains a hemoglobin level around 10. What is the best way treat her condition?
A. Annual examinations
B. Immediate splenectomy
C. Regular transfusions to maintain HGB above 12.0
D. Splenectomy followed by anti-pneumococcal vaccination
E. Splenectomy with a cholecystectomy
The answer is A.
EXPLANATION: There is currently no treatment recommended for patients with HS with a compensated anemia. This patient has a slight anemia that is compensated and would not require transfusions, splenectomy, or other treatment or therapy at this time. Annual examinations would be appropriate.
A 29-year-old female complains of a two-month history of easy bruising. She describes that the bruising is located primarily on her shins, but has noted them on other areas as well. She also describes red freckles on her lower extremities. On exam, you note non-blanching and non-palpable purpura to both legs and petechiae. She denies recent illnesses and states that she has essentially been feeling fine. The PE is normal other than the skin findings. A platelet count of 118,000 is noted. What is the best therapy?
A. Glucocorticoids
B. Intravenous Immunoglobulin
C. Observation
D. Rituximab
E. Splenectomy
The answer is C.
EXPLANATION: Most ITP patients are diagnosed incidentally. Signs and symptoms of bleeding are important in determining whether any treatment is required. Patients with no bleeding and consistent platelet counts above 50,000 do not require treatment, and may be observed periodically. All other therapies are used for treatment if patients develop bleeding problems or platelet counts drop below 20,000. The treatment will depend on individual needs.
A 28-year-old male presents with a diagnosis of Hemophilia A. Which of the following would he be deficient in?
A. Christmas factor
B. Factor VII
C. Factor VIII
D. Factor IX
E. Von Wildebrand’s factor
The answer is C.
EXPLANATION: Hemophilia A is caused by a defective synthesis of Factor VIII. Factor IX and Christmas factor are deficiencies in Hemophilia B. Deficiency of factor VII can exacerbate bleeding issues, but are not typically seen in Hemophilia A. Von Willebrand’s factor is a loss of this platelet-binding multimer, and most patients will have enough factor VIII but not the ability to bind factor VIII. In very severe cases of vWF, factor VIII may become low enough to produce symptoms similar to those found in factor VIII deficiency.
Which of the following would raise your suspicions the most and likely warrant consideration of testing for an inherited thrombophilia?
A. a deep femoral vein deep vein thrombosis (DVT) after a flight from Mumbai, India
B. an iliac vein DVT after a round trip bus trip to Atlantic City and playing slots all day
C. any DVT after a total knee replacement
D. an upper extremity DVT after tripping falling down a flight of stairs
E. any DVT in a woman who recently started oral contraceptives and smokes
The answer is D.
EXPLANATION: DVTs most commonly arise from the deep femoral veins and iliac arteries, most commonly in patients who smoke and take oral contraceptives, after immobilizing surgeries, and/or after immobilization due to long periods of time seated, including but not limited to airplane flights, bus rides, etc. Upper extremity DVTs are rare, even after trauma, and warrant a hypercoaguability work-up to rule out inherited disease.
A 64-year-old male presents with fatigue, pallor, and dyspnea on exertion. He has noted this gradual onset over the last several months. His initial reason for coming in was due to new papules and nodules that he noted on his skin, with some violaceous in color. On exam you also note petechiae to the lower extremities and splenomegaly. On questioning him further, he notes feeling full sooner than he had in the past. On laboratory you note a pancytopenia. What is the most likely diagnosis?
A. Acute myelogenous leukemia with leukemia cutis
B. Hereditary Spherocytosis with drug eruption
C. Lymphoma with erythema nodosum
D. Pyoderma Gangrenosum
E. Sarcoidosis
The answer is A.
EXPLANATION: The most likely diagnosis is AML with leukemia cutis. Leukemia cutis is seen in a subset of AML patients that are nontender, and are infiltrates of leukemic cells into the dermis. Hereditary spherocytosis is a hemolytic anemia, and does not present with papules and nodules or pancytopenia. Lymphoma does not typically present with a pancytopenia or skin rash. Pyoderma gangrenosum is an uncommon ulcerative cutaneous condition of uncertain etiology. Sarcoidosis is a chronic noncaseating granulomatous disease of unknown etiology, which affects many organs and tissues, most commonly the lungs.
A 27-year-old female presents to the emergency department with severe RUQ pain. Ultrasonography reveals gallstones. In preparation for a potential cholecystectomy, a CBC is obtained that reveals a normocytic anemia with an increased mean corpuscular hemoglobin concentration (MCHC). She is slightly jaundiced and you are able to palpate her spleen on examination. What is her underlying diagnosis?
A. Hereditary spherocytosis
B. Iron deficiency anemia
C. Sickle cell anemia
D. Thalassemia
E. Von Willebrand’s disease
The answer is A.
EXPLANATION: Hereditary Spherocytosis (HS) is characterized by jaundice, an enlarged spleen, and often gallstones; gallstones are more frequently seen in young people, triggering the investigation into HS. An increased MCHC is a characteristic feature of HS and is almost the only condition in which this finding is seen. Iron deficiency anemia does not have an increased MCHC. Sickle Cell has characteristic findings different than the presenting findings. Thalassemia is noted for a microcytosis and Von Willebrand’s is a coagulation disorder.
A 41-year-old alcoholic male, who lives primarily on the streets, appears pale, cachectic, and mildly icteric. He is complaining of several weeks of increasing fatigue. Laboratory findings note an elevated MCV of 128. What other physical finding would most support the diagnosis for megaloblastic anemia?
A. Decreased vibration and position sense
B. Dementia
C. Difficulty with balance
D. Glossitis
E. Parethesias
The answer is D.
EXPLANATION: Features of folate deficiency are similar to vitamin B12 deficiency. However, there are none of the neurologic abnormalities associated with vitamin B12. Glossitis is the only non-neurologic finding in the PE that would support folate deficiency. Alcoholism and poor dietary intake also support the diagnosis of folate deficiency.
A newborn male is diagnosed with Christmas factor deficiency. What is the likelihood that he inherited this disorder from his father?
A. 0%
B. 25%
C. 50%
D. 75%
E. 100%
The answer is A.
EXPLANATION: All daughters of a hemophilic male are carriers of hemophilia, whereas all sons are normal. Hemophilia B (or Christmas factor deficiency) is one of only two sex-linked pattern-bleeding disorders, and as such the disease occurs almost exclusively in males. Sons of carriers have a 50% chance of being affected and daughters of carriers have a 50% chance of being carriers themselves.
A 17-year-old female (Ht: 5’6”, Wt: 105 lbs) is noted to have macrocytic anemia. Her serum vitamin B12 level is 298 (normal range 203 to 339). What is the most likely diagnosis?
A. Folate deficiency
B. Hemolytic anemia
C. Iron deficiency anemia
D. Sideroblastic anemia
E. Vitamin B12 deficiency
The answer is A.
The most common cause of folate deficiency is inadequate dietary intake. This patient is anorexic, which increases her chances of developing a folate deficiency, a common cause of macrocytic anemias. Her vitamin B12 level is in the normal range, making this diagnosis unlikely. Iron deficiency causes a microcytic anemia. Sideroblastic anemia is diagnosed by examining the bone marrow, and the MCV is usually normal. Hemolytic anemias generally do not affect MCV.
A 74-year-old male has been diagnosed with aplastic anemia. His temperature is 37.4°C, heart rate is 68, respiratory rate is 20, and blood pressure is 130/86. Mild petechiae are noted to the extremities and there is some scattered bruising. The patient is currently taking Captopril, HCTZ, Lipitor, and Flo-max. Laboratory findings include:
WBC: 2.9
HGB: 10.4
PLT: 54,000
Initial management for this patient should include which of the following?
A. Broad spectrum antibiotics
B. Discontinuation of Captopril
C. Immediate assessment for allogeneic stem cell transplant
D. Irradiated and leukocyte depleted red cell transfusion
E. Platelet transfusion
The answer is B.
EXPLANATION: While the laboratory findings demonstrate a pancytopenia, levels are not low enough or physical findings are not suggestive of the need for transfusions at this time. Given the patients age, he is not eligible for an allogeneic stem cell transplant. He has no physical findings suggestive of a systemic infection requiring antibiotics. Captopril has a low risk of causing pancytopenias. It is best to discontinue this drug first as a possible cause of the pancytopenia, while continue to treat this patient symptomatically
Which of the following lab test is considered the “gold standard” for detecting/diagnosing an inherited thrombophilia?
A. anticardiolipen deficiency
B. factor V Leiden deficiency
C. hyperhomocysteinemia
D. protein C and S deficiency
E. There is no single lab test.
The answer is E.
EXPLANATION: There is no single “gold standard” lab test for diagnosis of acquired/hereditary thrombophilia.
A 9-year-old male presents with an acute onset of petechiae, ecchymoses, and gingival bleeding. He has pallor, fatigue, and bony pain. A pancytopenia is noted on CBC. Ebstein-Barr is negative. Lymphoblasts are noted on smear. Vitals reveal a temperature of 100.8F, HR 74, and RR 20. A few shoddy cervical nodes are noted. What is the most likely diagnosis?
A. Acute lymphoblastic leukemia
B. Aplastic anemia
C. Infectious lymphocytosis
D. Infectious mononucleosis
E. Lymphoma
The answer is A.
EXPLANATION: This patient has acute lymphoblastic leukemia (ALL). While all the diseases would be in the differential, only ALL fits all the findings. Infectious mononucleosis is excluded with a negative EBV. Infectious lymphocytosis would not have a pancytopenia or increased blasts on smear. Aplastic anemia would have a pancytopenia but not bony pain. Lymphoma typically does not have a pancytopenia associated with the disease.
A 68-year-old female presents with symptoms of kidney failure. Her creatinine is elevated at 1.9, and monoclonal light chains are seen in the urine. She complains of fatigue and bony pain that has been increasing over the last several months. This patient is most likely experiencing symptoms from which disease?
A. Acute myelogenous leukemia
B. Chronic myelogenous leukemia
C. Hodgkin lymphoma
D. Multiple myeloma
E. Non-Hodgkin lymphoma
The answer is D.
EXPLANATION: 30 to 50% of patients presenting with multiple myeloma will have some form of renal impairment. The most frequent cause is the formation of myeloma cast nephropathy. This is due to formation of tubular casts in the distal nephron, formed by the binding of light chains to uromodulin. These tubular casts obstruct the distal nephron and parts of the ascending loop of Henle, and contribute to development of interstitial nephritis. Light chains are not seen in the urine of any of the other diseases listed.
A 57-year-old male is being monitored for Binet Stage A CLL. He is emergently seen in the clinic with rapid lymph node enlargement, fever, weight loss, and hepatosplenomegaly. On laboratory examination, he is found to have an elevated serum lactate dehydrogenase and a monoclonal gammopathy on serum protein electrophoresis. A retroperitoneal ultrasound reveals bulky adenopathy. What is the most likely diagnosis?
A. Acute lymphadenitis secondary to HSV
B. Epstein-Barr viral infection
C. Hodgkin lymphoma
D. Rapidly advancing CLL
E. Richter transformation
The answer is E.
EXPLANATION: All findings are classic for Richter transformation of CLL to an aggressive large B-cell, high-grade lymphoma. While HSV cannot be totally ruled out without a node biopsy, given the patient history and findings the most likely diagnosis is Richter transformation. Rapidly advancing CLL does not usually develop retroperitoneal adenopathy. Occasionally, Richter syndrome with Hodgkin lymphoma features is seen, but accounts for less than one-fifth of all cases of Richter transformation. EBV may play a role in this later syndrome.
A 4-year-old is diagnosed with severe thrombocytopenia, causing spontaneous bleeding two weeks after having influenza. The child has no evidence of anemia, neutropenia, or anything else that raises a suspicion for an alternate diagnosis, and there are no atypical findings on the blood film. What is the most likely diagnosis?
A. Autoimmune hemolytic anemia
B. Immune thrombocytopenic purpura
C. Thalassemia
D. Thrombotic thrombocytopenic purpura
E. Von Willebrand’s disease
The answer is B.
EXPLANATION: In children with no other potential causes, the most likely diagnosis is immune thrombocytopenic purpura. The disease must be distinguished from acute lymphoblastic leukemia in this population prior to finalizing the diagnosis. Autoimmune hemolytic anemia would present with an anemia. Thalassemia is an inherited hematologic disorder, von Willebrand is a bleeding disorder typically with normal platelet counts, and thrombotic thrombocytopenic purpura typically has an associated anemia and a lack of bleeding.
A patient that has exhibited excessive bleeding tendencies is scheduled for a cholecystectomy. By history, he is noted to be of Ashkenazi Jewish decent. Which coagulation disorder is most likely the cause of his bleeding tendency?
A. Factor V
B. Factor VII
C. Factor VIII
D. Factor IX
E. Factor XI
The answer is E.
EXPLANATION: Most patients with factor XI deficiency are Jewish, with most in the Ashkenazi population. While other factors may affect individual patients, the most common of these coagulation disorders within this population base is factor XI.
A 31-year-old female presents with headache, fever, and petechiae to her lower extremities. A microangiopathic hemolytic anemia is noted. She denies diarrhea, recent infectious episodes, cough, shortness of breath, or urinary symptoms. What other finding would you expect to find in this woman to confirm the diagnosis?
A. Blasts on blood smear
B. Edema
C. Hepatomegaly
D. Thrombocytopenia
E. Sickle Cell
The answer is D.
EXPLANATION: This patient has classic signs of thrombotic thrombocytopenic purpura. Microangiopathic hemolytic anemia and thrombocytopenia are seen in all cases of TTP in the absence of another plausible explanation. Edema is not seen in TTP. Sickle cell is found in sickle cell anemia. Hepatomegaly is seen in less than 20% of cases of TTP and does not confirm the diagnosis. Blasts are seen in leukemia and do not confirm the diagnosis.
A 38-year-old male presents with red-brown urine in the mornings. The discoloration has come and gone several times over the last several months. He has also noted hard, painful areas on his skin, which seem to be located over veins, which have occurred intermittently. Laboratories reveal nonspherocytic red cells and a Coombs negative intravascular hemolysis. What is the most likely diagnosis?
A. Acute Myelogenous Leukemia
B. Aplastic Anemia
C. Iron Deficiency Anemia
D. Paroxysmal Nocturnal Hemoglobinuria
E. Von Willebrand’s Disease
The answer is D.
EXPLANATION: Urine that appears to be bloody, primarily in the morning and episodic in nature, along with thrombosis of dermal veins, nonspherocytic red cells and Coombs negative intravascular hemolysis are classic signs of PNH. PNH may be a symptom of aplastic anemia, but this patient doesn’t have a pancytopenia. He also does not have a microcytic anemia, which rules out iron deficiency. Von Willebrand’s is a coagulation disorder.
During a pre-surgical workup, a patient reports that she had received Desmopressin following the birth of her son. Her CBC is within normal range, as well as her PT and aPTT. What is the most likely cause of her needing this medication?
A. Allo immunization following vaginal delivery
B. Hemolytic anemia
C. Iron deficiency anemia
D. Thalassemia
E. Von Willebrand’s disease
The answer is E.
EXPLANATION: Desmopressin is the mainstay of therapy for people with von Willebrand’s disease. Hemolytic anemia, iron deficiency anemia, and thalassemia would all have abnormal findings on the CBC. Allo immunization does not require desmopressin therapy.
A patient that was diagnosed with severe hemophilia B in 1974 and requiring regular factor IX replacement therapy will have a 1:2 chance of also having which of the following?
A. Hemarthroses
B. Hemophilia A
C. Hepatitis B
D. Hepatitis C
E. HIV
The answer is E.
EXPLANATION: Approximately 50% of older and severely affected patients with either hemophilia A or hemophilia B are now HIV positive, due to the pooled nature of therapeutic products prior to 1985. These patients are also at increased risk for Hepatitis B and C, but not at the same ratio. Hemarthroses is a complication of not treating factor IX bleeding.
This common malignancy is diagnosed in patients younger than 15 years of age, has an incidence peak during early childhood (2 to 4 years old), and is seen more prominently in industrialized nations. What is the name of this malignancy?
A. Acute Lymphoblastic Leukemia
B. Acute Myelogenous Leukemia
C. Chronic Lymphocytic Leukemia
D. Chronic Myelogenous Leukemia
E. Lymphoma
The answer is A.
EXPLANATION: Acute Lymphoblastic Leukemia is the most common malignancy diagnosed in patients younger than age 15, and has a peak incidence of 2 to 4 years of age. It is seen more often in males, and the pattern affected suggests a leukemogenic contribution from factors associated with industrialization.
A 27-year-old African American with sickle cell anemia presents to the emergency department with acute onset intractable pain. She is taking quick, shallow breaths and her oxygen saturation is 84% on room air. She appears desiccated, states she hasn’t eaten in the last 24 hours, and says that she “just doesn’t feel well.” She is also afebrile. What should your next course of action be?
A. Start morphine, hydrate, and start antibiotics
B. Start morphine, oxygen, and start antibiotics
C. Start oxygen, hydrate, and exchange transfusion
D. Start oxygen, hydrate, and give pneumococcal vaccination
E. Start oxygen, hydrate, and start antibiotics
The answer is C.
EXPLANATION: Start oxygen, hydrate, and exchange transfusion
Exchange transfusions are primarily indicated for the treatment of intractable pain crises, priapism, and stroke. Patients should be kept well hydrated, and oxygen should be given if the patient is hypoxic. Antibiotics would be used if there was an infection identified, but are not part of initial treatment in a sickle crises.
