HEME FINAL PREP

Auer rods on peripheral smear indicate...

AML

60 year old male with severe anemia, decreased neutrophil count, and small, abnormal B lymphocytes in the bone marrow (>30%) with levels at 90,000 per cubic millimeter. Has painless cervical lymphadenopathy and hepatosplenomegaly. Likely diagnosis?

CLL

Bone marrow aspiration and biopsy reveals the presence of the Philadelphia (Ph) chromosome in dividing marrow cells. Likely diagnosis?

CML

Reed Sternberg cell

Hodgkin's lymphoma

Plasma cells, monoclonal protein

MM

What disease is characterized by abnormal monoclonal protein, anemia, and lytic bone lesions?

MM

30 year old white male with 3 month history of painless enlargement of bilateral cervical lymph nodes, fever, and night sweats. Likely diagnosis?

Hodgkin's disease

4 year old with fever, bleeding, bone pain, generalized lymphadenopathy, splenomegaly, hepatomegaly. CBC shows anemia, leukocytosis (100,000 immature WBC) and thrombocytopenia. Prognosis is good. Likely diagnosis?

ALL

60 year old male with no symptoms and no abnormalities on physical exam has a CBC that shows hemoglobin of 9.0, leukocytes 40,000, and peripheral smear shows 97% small lymphocytes. Likely dx?

CLL

What are B symptoms?

Fever, drenching night sweats, fatigue, weight loss

Most common presentation for Non-Hodgkin's lymphoma

painless lymphadenopathy

What test excludes the diagnosis of polycythemia vera if it is suspected?

Elevated plasma erythropoietin level

What is the most common form of adult leukemia in Western countries?

CLL

What 2 types of leukemia may have the Philadelphia chromosome?

AML CLL

Smudge cells

CLL

What is the most deadly phase of chronic myelogenous leukemia?

Acute blast crisis phase (>30% blasts in blood or bone marrow)

What type of leukemia have lymphocytes with hairlike cytoplasmic projections?

Hairy cell leukemia

Hodgkin's lymphoma has a bimodal age distribution. When are they?

The first peak is in the second and third decades of life, the second peak is after the age of 50

What test will confirm your diagnosis of multiple myeloma?

Protein electrophoresis may show IgM proteins or Bence Jones proteins

A 55-year-old non-smoking male presents with a hemoglobin of 18.5 g/dl and a hematocrit of 56%. Which of the following physical examination findings is the most likely to be noted with this patient?

A. Splenomegaly

B. Cheilosis

C. Purpura

D. Decreased vibratory sense

(c) A. Patients with polycythemia vera present with elevated hemoglobin and hematocrit. On physical examination plethora, engorged retinal veins, and splenomegaly are common.

(u) B. Cheilosis is noted in iron deficiency anemia.

(u) C. Purpura is typically noted in bleeding disorders.

(u) D. Decreased vibratory sense is noted in vitamin B12 deficiency.

A 60-year-old presents with fatigue and splenomegaly. CBC reveals the following: WBC- 24,000/microliter, Hgb- 13.5 g/dl, Hct- 40%, MCV- 87 fL, MCHC- 34 g/dl, MCH- 28 pg, and platelets- 380,000/mL. The differential reveals neutrophils- 11%, lymphocytes- 80%, monocytes- 8%, and basophils- 1%. What is the most likely diagnosis?

A. Acute lymphocytic leukemia

B. Acute myelogenous leukemia

C. Chronic lymphocytic leukemia

D. Chronic myelogenous leukemia

(u) A. Acute lymphocytic leukemia is more common in children and presents with blasts in the peripheral blood.

(u) B. Acute myelogenous leukemia presents with pancytopenia and presence of blasts in the peripheral blood.

(c) C. Chronic lymphocytic leukemia presents with a WBC count greater than 20,000/microliter and absolute lymphocyte count of greater than 5000/microliter.

(u) D. Chronic myelogenous leukemia presents with elevated WBC count, marked left shift in the myeloid series of cells, and positive for Philadelphia chromosome.

A patient with multiple myeloma should be immunized against which of the following organisms?

A. Streptococcus pneumoniae

B. Hepatitis C virus

C. Listeria monocytogenes

D. Epstein Barr virus

(c) A. Patients with multiple myeloma are prone to infections with encapsulated organisms such as Streptococcus pneumoniae and Haemophilus influenzae.

(u) B. See A for explanation.

(u) C. See A for explanation.

(u) D. See A for explanation.

It is essential to advise a patient with infectious mononucleosis to

A. avoid contact sports.

B. continue to rest as much as possible.

C. gargle with salt water for relief of throat pain.

D. rest voice to prevent hoarseness.

(c) A. Patients with acute infectious mononucleosis often have enlargement of the spleen. Contact sports increase the risk for splenic rupture after contact. The other answers are suggestions that may improve symptoms but do not truly impact the care of the patient.

(u) B. See A for explanation.

(u) C. See A for explanation.

(u) D. See A for explanation.

Which of the following leukemias is most closely associated with the Philadelphia chromosome?

A. hairy cell

B. acute lymphocytic

C. acute myelocytic

D. chronic myelocytic

D) CML

A 26 year-old male presents with a four week history of fatigue, night sweats, and a painless mass in his neck. Physical examination confirms the presence of an enlarged right posterior cervical lymph node. What is the next best step in the evaluation of this patient?

A. bone marrow biopsy and aspiration

B. lymph node biopsy

C. chest CT scan

D. reexamine in 2-4 weeks

(u) A. See B for explanation.

(c) B. This patient presents with possible lymphoma. Diagnosis is made by lymph node biopsy. Bone marrow biopsy and CT scan of the chest are used for staging of the disease.

(u) C. See B for explanation.

(u) D. With the presence of B-symptoms (fever, night sweats, and weight loss) the patient needs a biopsy to evaluate for possible lymphoma.

A 56 year-old male is noted to have a recent diagnosis of polycythemia vera. His current hemoglobin is 21 gms/dl. What treatment should be instituted for this patient at this time?

A. Phlebotomy

B. Iron chelation therapy

C. Bone marrow radiation therapy

D. Normal saline IV hydration

(c) A. This patient has a diagnosis of polycythemia vera treatment begins with phlebotomy instituted on a weekly basis until the hematocrit is less than 45%. Maintainance of the hematocrit at 45% is achieved with repeated phlebotomy as necessary.

(u) B. See A for explanation.

(h) C. Radiation therapy has no place in this treatment.

(h) D. IV hydration with normal saline will place the patient into volume overload.

A 48 year-old male complains of weakness and general malaise for about 2 months. Patient denies any recent illness and does not take any medications. Physical examination reveals a pale looking male in no acute distress. His heart rate is 110 beats/minute without a murmur and his abdominal examination reveals hepatosplenomegaly. A CBC reveals the WBC to be 62,000/microliter, Hgb is 8.3 gms/dl, Hct is 24.6%. A differential reveals a predominance of monoblasts and promyelocytes with Auer rods present. What is the most likely diagnosis?

A. Acute lymphocytic leukemia

B. Acute myelogenous leukemia C. Chronic lymphocytic leukemia

D. Chronic myelogenous leukemia

(u) A. Acute lymphocytic leukemia (ALL) is a leukemia most often seen in children with lymphoblasts predominating. Adults who develop ALL usually have a prolymphocytic cell presentation.

(c) B. Acute myelogenous leukemia (AML) is a leukemia that presents in adults with a cell lineage of the immature granulocytic cells seen in the peripheral blood e.g. myeloblasts, promyelocytes. Auer rods are commonly seen in this condition.

(u) C. Chronic lymphocytic leukemia (CLL) is a leukemia that presents in older adults with WBC counts up to 100,000/mm3. On peripheral blood smears the cell that predominates is a mature lymphocyte.

(u) D. Chronic myelogenous leukemia (CML) is a leukemia that presents in adults with a cell lineage of mature cell lines with a marked increase in basophils and eosinophils. These cells are also known for having the Philadelphia chromosome.

Ref: (28)

A patient receiving heparin therapy for 6 days for deep vein thrombosis develops thrombosis at the IV site. The INR is 1.1. The aPTT is 66 seconds and the platelet count is 47,000 down from 148,000 on admission. Which of the following is the most appropriate treatment?

A. Protamine sulfate administration

B. Platelet administration

C. Discontinue the heparin

D. Schedule the patient for plasmapheresis

(u) A. See C for explanation.

(u) B. See C for explanation.

(c) C. Once the diagnosis of heparin induced thrombocytopenia is suspected treatment should include immediate discontinuation of all forms of heparin and treatment with a direct thrombin inhibitor should begin. Platelet administration is generally not indicated. Protamine sulfate is used for heparin overdosage and plasmapheresis would not be indicated as the platelet count will resume when the heparin is discontinued.

(u) D. See C for explanation.

A 52 year-old female is diagnosed with chronic myeloid leukemia (CML). Which of the following would you expect to find on physical examination of this patient?

A. Hepatomegaly

B. Splenomegaly

C. Posterior cervical lymphadenopathy

D. Inguinal lymphadenopathy

(u) A. See B for explanation.

(c) B. CML patients present with an enlarged spleen.

(u) C. CML patients typically present with enlarged spleen and fatigue, but there are not enlarged lymph nodes.

(u) D. Patients with CML do not present with inguinal lymphadenopathy.

A 27 year-old male nonsmoker is diagnosed with upper extremity venous thrombosis. What is the most likely factor abnormality found in this patient?

A. Factor VIII

B. Factor V

C. Factor I

D. Factor X

(u) A. See B for explanation.

(c) B. In young adults with otherwise unexplained thrombosis, factor V Leiden is the most common associated abnormality.

(u) C.See B for explanation.

(u) D.See B for explanation.

Which of the following is one standard of care to treat a first-time deep vein thrombosis (DVT) without pulmonary embolism (PE)?

A Begin the patient on warfarin 5 mg PO once daily and check an INR in five days and adjust to therapeutic levels continuing warfarin for six months.

B Begin the patient on enoxaparin 1 mg/kg SQ QD while also starting warfarin 5 mg PO once daily and check INR in five days and adjust to therapeutic levels continuing warfarin for six months and enoxaparin until therapeutic on the warfarin.

C Begin the patient on enoxaparin 2 mg/kg SQ QD while also starting warfarin 5 mg PO once daily and check INR in five days and adjust to therapeutic levels continuing warfarin for six months and enoxaparin until therapeutic on the warfarin.

D Begin the patient on heparin sodium IV and monitor and adjust levels to achieve therapeutic levels Q six hours and transition to warfarin as soon as possible.

E Initiate a hypercoaguability work up and treat accordingly.

Patients with deep vein thrombosis (DVT) without pulmonary embolism (PE) do not necessarily require hospitalization. Outpatient therapy would include treatment with a low molecular weight heparinoid (LMWH), such as enoxaparin, subcutaneously while the patient becomes therapeutic on oral warfarin. The LMWH can be discontinued once therapeutic on warfarin, with continued monitoring of INRs for six months before considering discontinuation.

A 64-year-old, nonsmoking male presents to the clinic with complaints of headache and dizziness. Initial labs reveal hemoglobin of 20.1 g/dl, hematocrit of 60%, and platelet count of 567,000.

Which of the following is the most likely diagnosis in this patient?

A Acute myeloid leukemia

B Chronic myeloid leukemia

C Chronic obstructive pulmonary disease (COPD)

D Multiple myeloma

E Polycythemia vera

Polycythemia Vera

E Polycythemia vera often presents with complaints related to increased red blood cell mass, such as, headache, fatigue, and dizziness with elevated red blood cell mass with leukocytosis and/or thrombocytosis noted on CBC.

Chronic hypoxia from conditions such as COPD (C) can also cause elevated RBC mass, but is unlikely in this non-smoker. The patient lacks white blood cell abnormality symptoms (e.g., recurrent infections, elevated white count) making acute myeloid leukemia (A), chronic myeloid leukemia (B), or multiple myeloma (D) unlikely diagnoses.

A 23-year-old male with a recent diagnosis of Non-Hodgkin's lymphoma presents complaining of swelling of the neck and face, cough, and dyspnea on exertion. On exam you note dilated neck veins.

Which of the following is the most likely diagnosis?

A Angioedema

B Carotid artery dissection

C Cushing's syndrome

D Myxedema

E Superior vena cava syndrome

E The classic presentation signs and symptoms of superior vena cava syndrome (SVCS) are present in this patient. Although the most common cancer associated with SVCS is lung cancer, it also occurs in Non-Hodgkin's lymphoma.

Angioedema (A), carotid artery dissection (B), Cushing's syndrome (C) and myxedema (D) can result in facial swelling, but should not lead to the presence of dilated neck veins.

A 73-year-old, male with a 30 pack-year smoking history presents to the clinic with complaints of headache and dizziness. Labs reveal a hemoglobin of 21.3 g/dl, hematocrit of 63%, and platelet count of 498,000. He tests positive for the JAK2 mutation. Which of the following is the most appropriate treatment for the patient's suspected diagnosis?

A Allopurinol

B Anagrelide

C Diphenhydramine

D Hydroxyurea

E Phlebotomy

E The first-line, and primary treatment for polycythemia vera is chronic phlebotomy.

Patients who fail to improve or don't tolerate chronic phlebotomy can be treated with hydroxyurea (D), with anagrelide (B) being a third-line treatment. Allopurinol (A) may be necessary to treat hyperuricemia and gout. Diphenhydramine (C) may be used as symptomatic treatment for pruritus that doesn't improve with phlebotomy.

A 73-year-old, male with a 30 pack-year smoking history presents to the clinic with complaints of headache and dizziness. Labs reveal a hemoglobin of 21.3 g/dl, hematocrit of 63%, and platelet count of 498,000. He tests positive for the JAK2 mutation.

Which of the following should be recommended to this patient to prevent secondary complications from his diagnosis?

A Aspirin 81 mg daily

B Ferrous Sulfate 325 mg twice daily

C Eat a diet rich in vitamin B12

D Eat a diet rich in folate

E Maintain stable consumption of foods that contain vitamin K

Aspirin 81 mg daily

A The patient most likely has polycythemia vera and is at risk for thrombotic events that can be reduced through the use of daily aspirin. In addition, he should be counseled on smoking cessation if he is still currently smoking.

The patient should not take iron supplements (B) as they compete with phlebotomy therapy that seeks to reduce iron stores. Patient's with PCV similarly do not require vitamin B 12 (C) or folate (D) supplementation, and are not impacted by fluctuations in vitamin K consumption (E) unless they are being treated with warfarin for a current/recent thrombotic event.

A 22-year-old non-obese female presents to the clinic with sign and symptoms of deep vein thrombosis (DVT). She denies recent trauma, prolonged immobilization, smoking, use of oral contraceptives, or changes in her health. Her last menstrual period was one week ago. Which of the following is the most likely diagnosis that contributed to the development of her DVT?

A Antithrombin deficiency

B Factor V Leiden

C Protein C deficiency

D Protein S deficiency

E Hyperhomocysteinemia

Factor V Leiden

B Factor V Leiden mutation is one of the most common genetically inherited prothrombotic states.

Deficiencies of antithrombin (A), protein C (C) and protein S (D) when homozygous in nature typically present as fetal death or severe thrombosis at birth. When heterozygous, they carry a much lesser risk of developing thrombosis as compared to Factor V Leidin. Hyperhomocysteinemia (E) also carries a much lesser risk of developing thrombosis.

A 32 year-old obese woman presents to the clinic complaining of swelling, warmth, and redness of her left lower extremity. Doppler ultrasound confirms a deep venous thrombosis is the greater saphenous vein. Which of the following best describes the most likely pathophysiology underlying her thrombophilia?

A Antithrombin deficiency

B Increased plasma prothrombin

C Protein C deficiency

D Protein S deficiency

E Resistance to activated protein C

Resistance to activated protein C

Factor V Leiden is a common genetic mutation that results in resistance to activated protein C.

Increased plasma prothrombin concentration results from a prothrombin gene mutation (B), while deficiencies of antithrombin (A), protein C (C) and protein S (D) are rarer causes of prothrombotic states and are not directly associated with factor V Leiden mutation which is the most common cause of thrombophilia in this clinical scenario

A 23-year-old male with a recent diagnosis of Non-Hodgkin's lymphoma presents complaining of swelling of the neck and face, cough, and dyspnea on exertion. On exam you note dilated neck veins.

Which of the following is the most appropriate initial treatment for this patient?

A Beta-blockers

B Glucocorticoids

C IV fluids

D Trendelenburg position

E Vena cava stenting

Glucocorticoids

The classic presentation signs and symptoms of superior vena cava syndrome (SVCS) are present in this patient. Patients with lymphoma often respond with a decrease in tumor mass and improvement in SVCS with glucocorticoid therapy.

Beta-blockers (A) and vena cava stenting (E) aren't routinely used in the management of SVCS. Other initial symptomatic therapies include low salt diet, diuretics, head elevation, and diuretics. IV fluids (C) and Trendelenburg position (D) are contraindicated.

A 68-year-old female has been having increasing low back pain that radiates into her right hip and down her right leg. She has failed physical therapy and anti-inflammatories. She feels like the bones "are rubbing on each other." Radiographic imaging of the spinal cord, hip, and leg reveal several lytic lesions. The most helpful finding to confirm the diagnosis would include which of the following?

Plasmocytosis in the marrow

Plasmacytosis in the marrow is the most prominent and diagnostically important finding. Reed-Sternberg cells are seen in Hodgkin's lymphoma. Granulocytosis is an increased white cell count seen in leukemias. Macrocytosis is enlargement of red cells, and can be seen in vitamin B12 deficiency. Monocytosis is an increase in monocytes, and can be seen in infectious mononucleosis or some leukemias.

A 72-year-old male presents with bony pain, Bence Jones protein in his urine, an elevated creatinine level of 2.0, hypercalcemia, and lytic lesions to the long bones of his legs. The best initial treatment to correct this patient's renal insufficiency is which of the following?

Hydration and Calcitonin

Management of renal impairment in patients with multiple myeloma is primarily supportive care. Hydration and use of calcitonin is the mainstay. If the hypercalcemia needs rapid correction, a slow infusion of bisphosphonate may be used. Dialysis would be useful if the patient has renal failure but has difficulty removing light chains from the blood, which are causing the problem. Plasmaphoresis doesn't play a role in this setting.

A 58-year-old female two days status-post coronary artery bypass graft (CABG) surgery is being treated with heparin to prevent thrombosis. Her routine CBC reveals a hemoglobin of 11.2 mg/dL, hematocrit of 35%, WBC count of 5.6, and platelet count of 22,000. In addition to discontinuing heparin, which of the following is the most appropriate intervention?

A Administer prednisone

B Administer warfarin

C Bone marrow aspirate

D CT of the abdomen

E Observation

Administer Warfarin

The patient most likely has developed heparin-induced thrombocytopenia (HIT), which is associated with qualitative platelet function changes that result in increased risk of thrombosis. She should begin warfarin (B) and be evaluated for thrombosis (e.g., lower extremity Dopplers).

Prednisone (A) is not indicated for the treatment of HIT, and a bone marrow aspirate (C) would not aid in establishing the diagnosis. CT of the abdomen (D) would be indicated if she had symptoms consistent with thrombosis in that region, and observation (E) fails to address her increased thrombotic risk.

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 correct answer is (E).

There is no single "gold standard" lab test for diagnosis of acquired/hereditary thrombophilia.

A 70-year-old African American male was admitted to the hospital with complaints of a high grade fever, nausea, infection and severe back pain. Nearly two years prior while walking down his steps, he fell without incurring any apparent fractures. Since then he has been complaining of mild back pain, which worsened during cough. Lab reports reveal low red blood cell count but normal neutrophil and platelet count. The serum protein electrophoresis reveals the presence of paraprotein. Bone marrow biopsy reveals 50% infiltration by plasma cells. Based on this information, what would be your diagnosis?

Answer Choices

1 multiple myeloma

2 Waldenström's macroglobulinemia

3 monoclonal gammopathy of unknown significance (MGUS)

4 primary amyloidosis

5 polyclonal hypergammaglobulinemia

Multiple Myeloma

Explanation Multiple myeloma is a malignant disorder of the plasma cells. The disease occurs very frequently in people above 60 years of age. Males are more frequently affected than females. In the patients suffering from multiple myeloma, bone marrow biopsy shows heavy penetration with atypical plasma cells. Anemia is nearly universal in these patients. The hallmark of myeloma is the finding of a paraprotein on serum protein electrophoresis (SPEP). The majority of patients have a monoclonal spike in the beta or gamma globulin region. Overall, approximately 60% of myeloma patients have an IgG paraprotein, 25% have an IgA, and 15% have light chains only.

Monoclonal gammopathy of unknown significance (MGUS) closely matches multiple myeloma in the immunoelectrophoresis patterns. Thus, if one considers all patients with paraproteins, MGUS is far more common than myeloma. However, myeloma is distinguished from MGUS by findings of replacement of the bone marrow, bone destruction and progression over time. Although the height of paraprotein spike alone is not used to distinguish benign from malignant conditions. In practice all the patients with IgG spikes greater than 3.5g/dL prove to have myeloma. In polyclonal hypergammaglobulinemia there is a polyclonal instead of a monoclonal spike.

A 64-year-old, nonsmoking male presents to the clinic with complaints of headache and dizziness. Initial labs reveal hemoglobin of 20.1 g/dl, hematocrit of 60%, and platelet count of 567,000.

Which of the following laboratory finding confirms the suspected diagnosis?

A Bone marrow hypocellularity

B Elevated ferritin

C Elevated serum erythropoietin

D Presence of Philadelphia chromosome

E Presence of JAK2 mutation

E presence of JAK2 mutation

The clinical presentation is most consistent with polycythemia vera (PCV), which, in the presence of a markedly elevated red blood cell mass, is confirmed by the presence of the JAK2 mutation.

The patient's erythropoietin (C) and ferritin (B) are most likely decreased as a result of bone marrow overproduction of RBCs and use of iron stores to produce these RBCs. The bone marrow of patients with PCV is typically hypercellular (A) across all cell lines.

A patient presents with hyperleukocytosis (WBC 100,000). The bone marrow is consistent with T-cell ALL. The uric acid is less than 7.0 mg/dl. Prior to starting chemotherapy, what is the best therapy to initiate on this patient?

A Allopurinol and IV fluids

B Aluminum hydroxide and calcium carbonate

C Glucocorticoids and vincristine

D Leukapheresis and cranial irradiation

E Sevelamer and mercaptopurine

A Allopurinol and IV fluids

Hyperuricemia is often a finding in patients with hyperleukocytosis. The optimal treatment is to start IV fluids, due to numbers of circulating white cells and allopurinol, to treat hyperuricemia. The use of aluminum hydroxide is appropriate if they have hyperphosphatemia, and calcium carbonate if they have a low serum calcium concentration. Glucocorticoids and vincristine are used with hyperleukocytosis of >400,000. Leukapheresis and cranial irradiation are used for patients with extreme leukocytosis of >400,000. Sevelamer is used to treat hyperphosphatemia, and mercaptopurine is a byproduct produced during production of leukemic cells.

A 38-year-old thin, HIV+ male is found to have a distended abdomen and is complaining of a feeling of fullness. A CT scan of the abdomen revealed a large abdominal mass with ascites in the retroperitoneal region. Laboratories found an elevated EBV of 5400 copies (normal=0), LDH 554 (100 to 250), and Ig-positive MYC translocation. What is the most likely diagnosis?

A Burkitt lymphoma

B Cat scratch disease

C Hodgkin's lymphoma

D Infectious mononucleosis

E Sarcoidosis

Burkitt Lymphoma

In immunosuppressed individuals, including those who are HIV+ and in the presence of highly reactive EBV, the development of an abdominal mass with elevated LDH is most likely Burkitt's lymphoma. Of the masses that are found, 30 to 40% will be positive for EBV, and it appears that this virus drives the conversion of cells to a cancerous state. Infectious mononucleosis would be positive for EBV, but does not develop large abdominal masses.