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  • 1. Practice A QUESTION

    Sample Question 1

    A 65-year-old man presents with a 2-week history of progressive dyspnea, orthopnea, and pedal edema. His history is significant for heavy smoking (30 cigarettes/day for more than 40 years), a 20 pound weight loss over past 3 months, loss of appetite, and weakness. His physical examination reveals pulse - 130 per min., blood pressure - 105/90 mmHg, paradoxic pulse, distant heart sounds, nonpalpable cardiac impulse, slight dullness at both lung bases, and bilateral pedal edema. His chest X-ray shows enlarged cardiac silhouette and a large pericardial effusion; they are confirmed by echocardiography. Acid-fast staining of the pericardial fluid is negative for Acid-Fast Bacilli (AFB). CT scan reports are awaited.


    What is the most likely etiology of this pericardial effusion?
     

    Answer Choices

    A. Pulmonary TB
    B. Viral pericarditis
    C. Bacterial pericarditis
    D. Malignant pericardial effusion
    E. Congestive heart failure

    2. Review ITS EXPLANATION

    The most likely etiology is a malignant pericardial effusion. Pericardial effusion is present in 20% of cancer patients. Cancer is the most frequent cause of pericardial tamponade, accounting for 16 to 41% of cases. This patient's smoking history, age, loss of appetite, and weight loss are strong predictors of malignant etiology. Lung and breast cancers are the most common cancers involving the pericardium; they are followed by leukemia, lymphoma, sarcoma, and melanoma. Unlike this case, malignant pericardial effusions are usually asymptomatic. When present, symptoms are nonspecific and can be obscured by symptoms of the malignancy. Only 30% of the effusions are correctly diagnosed ante-mortem. The diagnosis is most often suggested by enlarged cardiac silhouettes on chest X-ray and is confirmed by echocardiography. Cytology of the pericardial fluid for malignant cells can be performed. Pericardial biopsy in cases of malignant pericardial effusion is positive in only 55% of patients. Cardiac tamponade needs prompt treatment with pericardiocentesis to relieve the increased end-diastolic pressure and inadequate ventricular filling.

    Pulmonary TB is suspected if the X-ray shows mottled shadows, particularly in the upper lobes. Acid-fast staining will be positive for AFB.

    Viral pericarditis and bacterial pericarditis present chest pain over the anterior chest. As the patient inhales, the pain exacerbates and it is usually associated with tachycardia. A low-grade fever may also be present.

    Congestive heart failure is characterized by weakness, breathlessness, abdominal discomfort, and edema in lower portions of the body. The chest X-ray may show cardiac enlargement via increased size of the left ventricular shadow. There may be pulmonary congestion with audible rales. Echocardiography confirms the diagnosis.
    1. Refaat MM, Katz WE. Neoplastic pericardial effusion. Clin Cardiol. 2011 Oct. 34(10):593-8.



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  • 1. Practice A QUESTION

    Sample Question 2

    A 47-year-old woman presents for a routine physical exam. She notes that, in the last 5 months, she finds herself more easily fatigued; she has lost 25 pounds. In addition, the patient has recently noticed increasing pain in her arms and hip for the past 2 weeks. Physical examination is remarkable only for splenomegaly.

    Results from a blood count reveal marked leukocytosis (WBC = 52 x 109/L) with hemoglobin- 10.4 g/dl, hematocrit of 19.6%, and elevation of platelet count. Peripheral smear shows predominance of neutrophils and band cells as well as presence of myelocytes, metamyelocytes, and rare basophils. The differential count reveals 50% neutrophils, 20% band forms, 12% myelocytes, 6% metamyelocytes, 1% blasts, 6% lymphocytes, 2% basophils, and 3% eosinophils. Bone marrow aspiration findings reveal a hypercellular marrow with an increased M: E (myeloid: erythroid) ratio; maturation is preserved in the myeloid series although there is a left shift.


    The clinical picture and supporting laboratory data are consistent with what condition?
     

    Answer Choices

    A. Acute lymphoblastic leukemia (ALL)
    B. Chronic myelogenous leukemia (CML)
    C. Acute myelocytic leukemia (AML)
    D. Refractory anemia (RA)
    E. Refractory anemia with ringed sideroblasts (RARS)

    2. Review ITS EXPLANATION

    Chronic myelogenous leukemia (CML) is a clonal disorder of hematopoietic stem cells. Chromosomal abnormality commonly involved is the Philadelphia (Ph) chromosome. This leukemia accounts for approximately 15-20% of adult leukemia. The peak incidence is in the 4th and 5th decades of life. The median survival of patients with CML ranges between 4-5 years. The clinical course of CML is divided into three phases: chronic, accelerated, and the terminal blastic (blast crisis) phase. Generally, many patients come to medical attention during the chronic phase, often presenting with nonspecific symptoms such as weight loss or fatigue. Leukocytosis and splenomegaly are commonly seen. Progression of the condition to accelerated phase is marked by a clinical deterioration and an increase in symptoms such as fever and fatigue. The disease finally transforms into blast crisis having a phenotype markedly similar to acute leukemia. In some patients, there may be direct progression from chronic to blast phase. Blast crisis is generally resistant to standard chemotherapy and ultimately results in the death of the patient.

    Lab investigations such as the total white blood count (WBC) count are generally greater than 25,000 cells/?L and often range from 20,000-60,000 cells/?L. Other findings in CML include anemia, basophilia, and thrombocyctosis. The differential WBC count and peripheral blood smear (PS) often reflect the entire myeloid lineage (ie, presence of granulocytes at all levels of maturation). This diversity of circulating leukocytes, ranging from blasts to mature neutrophils, makes the peripheral blood smear similar in appearance to a typical bone marrow aspirate. Agents such as hydroxyurea or ?-interferon have been included in the standard approaches to therapy in order to control the peripheral blood counts. Bone marrow transplant (BMT) needs to be considered early in young patients having a matched sibling donor. Allogeneic BMT is the only curative therapy in CML.

    Acute myelocytic leukemia is a malignant disorder of the bone in which the hematopoietic precursors are arrested in an early stage of development. Consequently, the PS in AML shows leukemic gap (maturation arrest) or hiatus in which there is absence of different midstage progenitor cells. The presence of different midstage progenitor cells in CML differentiates it from AML.

    Refractory anemia and refractory anemia with ringed sideroblasts are a part of myelodysplastic syndrome (MDS). The clinical and lab findings in this patient are not consistent with RA or RARS.

    Acute lymphoblastic leukemia is a condition characterized by clonal proliferation of lymphoid precursor cells, replacing the normal hematopoietic cells of the bone marrow. In this condition the malignant cell is the lymphoid precursor cell (lymphoblasts), which is arrested in an early stage of development. There is anemia and thrombocytopenia of varying levels with low, normal, or high WBC count. The PS usually confirms the complete blood count findings and may also show lymphoblasts.

     
    1. DAVID P. STEENSMA, MD; ALAN F. LIST, MD; genetics in clinical practice: Genetic Testing in the Myelodysplastic Syndromes: Molecular Insights Into Hematologic Diversity; Mayo Clin Proc.2005; 80(5):681-698.
    2. Junia V. Melo, Timothy P. Hughes, and Jane F. Apperley, Chronic Myeloid Leukemia; Hematology 2003;2003: 132-152.
    3. Peter Maslak, M.D. Full Case Study; Chronic Myelogenous Leukemia American society of hematology Image Bank- 2001- 100202.
    4. Hill JM, Meehan KR. Chronic myelogenous leukemia: curable with early diagnosis and treatment. Postgrad Med 1999; 106(3):149-59.



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  • 1. Practice A QUESTION

    Sample Question 3

    A preterm infant develops abdominal distention and bilious vomiting. Meconium has not been passed since birth, which occured 2 days ago. Physical examination finds a distended abdomen. Rectal examination indicates increased sphincter tone and watery stool appears after the removal of examining finger. Plain radiograph shows absence of intra-intestinal air in the pelvis and a barium enema is inconclusive. What is the most appropriate next step in evaluating the likely diagnosis?
     

    Answer Choices

    A. Full-thickness rectal biopsy
    B. Trial with stool softeners
    C. Follow-up X-ray
    D. Anorectal manometry
    E. Repeat barium enema

    2. Review ITS EXPLANATION

    The clinical presentation is strongly suggestive of Hirschsprung's disease. It is a congenital condition which is characterized by the absence of ganglion cells in the submucosal (Meissner's) and myenteric plexus of the distal bowel. The failure of the ganglionic cells to fully migrate caudally during embryonic life is believed to be the cause. In Hirschsprung's disease anorectal manometry shows no recto-sphincteric inhibition reflex on rectal distension. Anorectal manometry is non-invasive and has good accuracy of detection; therefore, it is the diagnosis of choice in infants.

    Barium enemas help in establishing the diagnosis by identifying a transition zone between a narrowed aganglionic segment and a dilated normally innervated segment. However, a transition zone may not be apparent in neonates, because of insufficient time to develop colonic dilation, or in infants who have undergone rectal washouts, examinations, or enemas.

    Rectal biopsies have a high accuracy rate, and they are the definitive diagnosis, but it is an invasive method and it is shown that patients younger than 1 year have a higher risk of major complications. Seromuscular or full-thickness biopsy can detect the lack of Auerbach's plexus in the muscular layer. An experienced pathologist can make the diagnosis of Hirschsprung's disease by examining rectal suction biopsy specimen for the Meissner's plexus in the submucosal layer.

    The disease shows a strong male predominance. In most cases, the diagnosis is made within the first 2 years of life. In some cases, the history of constipation is prolonged and failure to thrive manifests. The absence of parasympathetic ganglia and consequential inability to relax require the removal of the involved intestine and establishment of functional anus with Swensen, Soave, or Duhamel procedure. Favorable response to stool softeners is associated with functional constipation, but they are not helpful in the case of Hirschsprung's disease.
    1. De Lorijn F, Kremer LC, Reitsma JB, Benninga MA. Diagnostic Tests in Hirschsprung Disease: A Systematic Review; J Pediatr Gastroenterol Nutr. 2006 May; 42(5):496-505.
    2. Lopez Alonso M, Hernandez Orgaz A, Ribas Serna J; Reliability index of anorectal manometry for the diagnosis of Hirschspurng disease; Cir Pediatr. 2005 Jan; 18(1):13-6.
    3. Harjai MM; Hirschsprung's Disease: Revisited; Journal of Postgraduate Medicine, Vol. 46, No. 1, January-March, 2000, pp. 52-54.



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    You can can now retain key concepts easily using our built-in Flashcards and Mnemonics.

    FLASHCARDS 2 MNEMONICS 10
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