Hematology/Oncology

Ep-PAINE-nym

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Blumer’s Shelf

Other Known Aliases – rectal shelf

Definition – shelf-like tumor of the anterior rectal wall (Pouch of Douglas) felt on rectal examination

Clinical Significance – palpation of this “shelf” indicates implantation metastases from primary abdominal malignancy

History – Names after George Blumer (1872-1962), who was an English-American physician and recieved his medical doctorate from the Cooper Medical College (forerunner of Stanford’s medical school) in 1891. He would go onto to train under William Halstead and William Osler at Johns Hopkins Hospital at house officer. In 1906, he became professor of medicine at Yale culminating in Dean of the medical school from 1910-1920. It was during this tenure (1909) when he described his eponymous finding in an article entitled “Rectal shelf: neglected rectal sign of value in diagnosis of obscure malignant and inflammatory disease within the abdomen”.

References

  1. Firkin BG and Whitwirth JA.  Dictionary of Medical Eponyms. 2nd ed.  New York, NY; Parthenon Publishing Group. 1996.
  2. Bartolucci S, Forbis P.  Stedman’s Medical Eponyms.  2nd ed.  Baltimore, MD; LWW.  2005.
  3. Yee AJ, Pfiffner P. (2012).  Medical Eponyms (Version 1.4.2) [Mobile Application Software].  Retrieved http://itunes.apple.com.
  4. Whonamedit – dictionary of medical eponyms. http://www.whonamedit.com
  5. Up To Date. www.uptodate.com
  6. Blumer G. Rectal shelf: neglected rectal sign of value in diagnosis of obscure malignant and inflammatory disease within the abdomen. Albany Medical Annals. 1909;30:361-366.
  7. Haubrich WS. Blumer of Blumer’s Shelf. Gastroenterology. 2000;118(1):30

#57 – Acute Myeloid Leukemia

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Epidemiology

  • Most common type of acute leukemia and the second most common type in adults
    • 32% of all adult leukemia cases
    • Only 1% of all adult cancers deaths in the US
      • Around 12,000 deaths per year in US
  • 3-5 cases per 100,000 population
  • Around 20,000 patients per year in the US get diagnosed
    • 2% annual increase in cases from 2007-2016
  • Mean age of diagnosis is 65 years and increases with age

Pathophysiology

  • Malignancy of myeloid precursor cells
    • Multipotential hematopoietic stem cell –> common myeloid progenitor –> myeloblast
  • 2 main models
    • Occurs at one of several developmental stages
    • Occurs within the primitive multipotent cells
  • Two-hit hypothesis of leukemogenesis
    • Class I mutation
      • Confers a proliferative advantage
    • Class II mutation
      • Impairs hematopoietic differentiation
  • Mechanisms of Genetic Damage
    • Chemotherapy
      • 3-5 years after alkylating agent-induced damage
    • Ionizing radiation
      • Induces double strand breaks
      • Typically > 20 Gy (grays)
    • Chemical exposure
      • Benzene is classically associated with AML
    • Infections
      • Human T-lymphocyte virus type I (HTLV-1

Signs and Symptoms

  • Constitutional
    • Fatigue, weakness, dyspnea
    • Fever
  • Skin (13% of patients)
    • Easy bruisability, ecchymoses
    • Pallor
  • HEENT
    • Gingival bleeding, oral candidiasis
    • Papilledema, retinal infiltrates
    • Lymphadenopathy
  • Abdomen (10% of patients)
    • Organomegaly
      • Spleen and liver
  • Musculoskeletal (4% of patients)
    • Polyarthritis and arthralgias
    • Bone pain

Emergency Presentations

  • Pancytopenia
  • Tumor lysis syndrome
    • Hyperkalemia, hyperphosphatemia, hyperuricemia, AKI
  • Bleeding
  • New onset CNS symptoms

Diagnostic Studies

  • CBC
    • Leukocytosis or leukopenia
      • 20% of patients have > 100,000 cells/microL
      • 25-40% of patients have < 5,000 cells/microL
    • Thrombocytopenia
      • 75% of patients have < 100,000 cells/microL
      • 25% of patient have < 25,000 cells/microL
  • Peripheral Blood
    • 95% of patients will have circulating myeloblasts
      • Immature cells with large, prominent nuclei and variable amount of pale blue cytoplasms
      • May have Auer rods present
    • Myeloperoxidase reaction
      • Simple means of determining if the blasts are myeloid
  • Flow Cytometry
    • Can assist in detecting circulating myeloblasts
  • Bone Marrow Biopsy
    • This is the key component in the diagnosis of AML
    • It gives a general overview of the degree of involvement, allows for cell differential count to determine the percentage of blasts in the marrow, and provides a detailed cytologic evaluation of the blasts
    • Cell Origin
      • Identifies if myeloid, monocytic, erythroid, or megakaryocytic
      • Differentiates the blasts of lymphoid lineage
    • Infiltration
      • Diagnosis of AML is > 20% blasts of the total cellularity
  • HLA Typing in patients who are potential candidates for bone marrow transplantation

Diagnosis

  • Requires both of the following criteria:
    • Documentation of bone marrow infiltration
      • > 20% blasts in bone morrow
    • Myeloid origin
      • Presence of Auer rods, (+) myeloperoxidase reaction, or presence of myeloid markers on immunophenotyping

Treatment

  • Goals
    • Complete remission (<5% blasts)
      • Appropriate goal for most AML patients
  • Pretreatment evaluation
    • Comorbid conditions
      • Heart disease, renal insufficiency, liver disease
    • Physical function and performance status
      • ECOG Scale most commonly used
  • Two distinct treatment phases
    • Induction
      • Combination therapy (7 and 3 regimen)
        • Cytarabine
          • Interferes with DNA synthesis
          • 7 day continuous infusion
        • Anthracycline
          • Daunorubicin, idarubicin
            • Inhibition of topoisomerase II
              • Leads to DNA breaks
          • Day 1, 2, and 3
      • Bone marrow biopsy 7-10 days after induction to re-assess
    • Postremission management
      • Continuing chemotherapy
      • Hematopoietic cell transplant

Prognosis

  • Overall 5-year survival is 15%
    • Decreases with age
      • 53% 5-year survival in 15-24yo to 13% in 70-79yo
  • Genetic subtypes
    • Karyotypes
    • Gene mutations

The Cottage Physician (1893)

References

  1. Acute myeloid leukemia statistics.  Cancer.net.  Accessed on 03/22/2020 [link]
  2. Blum W, Bloomfield CD. Acute Myeloid Leukemia. In: Jameson J, Fauci AS, Kasper DL, Hauser SL, Longo DL, Loscalzo J. eds. Harrison’s Principles of Internal Medicine, 20e New York, NY: McGraw-Hill; . http://accessmedicine.mhmedical.com.ezproxy.uthsc.edu/content.aspx?bookid=2129&sectionid=192017732. Accessed March 22, 2020.
  3. Siegel RL, Miller KD, Jemal A. Cancer Statistics, 2017. CA: a cancer journal for clinicians. 2017; 67(1):7-30. [pubmed]
  4. Reilly JT. Pathogenesis of acute myeloid leukaemia and inv(16)(p13;q22): a paradigm for understanding leukaemogenesis? British journal of haematology. 2005; 128(1):18-34. [pubmed]
  5. Levine EG, Bloomfield CD. Leukemias and myelodysplastic syndromes secondary to drug, radiation, and environmental exposure. Seminars in oncology. 1992; 19(1):47-84. [pubmed]
  6. Shuryak I, Sachs RK, Hlatky L, Little MP, Hahnfeldt P, Brenner DJ. Radiation-induced leukemia at doses relevant to radiation therapy: modeling mechanisms and estimating risks. Journal of the National Cancer Institute. 2006; 98(24):1794-806. [pubmed]
  7. Austin H, Delzell E, Cole P. Benzene and leukemia. A review of the literature and a risk assessment. American journal of epidemiology. 1988; 127(3):419-39. [pubmed]
  8. Shah A, Andersson TM, Rachet B, Björkholm M, Lambert PC. Survival and cure of acute myeloid leukaemia in England, 1971-2006: a population-based study. British journal of haematology. 2013; 162(4):509-16. [pubmed]

Ep-PAINE-nym

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Codman’s Triangle

Other Known Aliases – none

Definition – triangular area of new subperiosteal bone that is created when a bone tumor raises the periosteum away from the healthy bone

Clinical Significance – this occurs because the tumor is growing at a faster rate than the periosteum can expand, which leads to the periosteum tearing away and providing a second edge of ossification (thus making the triangle). Presence of this finding is highly suggested of a fast growing, malignancy.

History – Names after Ernest Amory Codman (1869-1940), who was an American surgeon and received his medical doctorate from Harvard University in 1895. Aside from being an accomplished surgeon, he fought for hospital reform and was an early adopter and advocate for patient-based outcomes. In fact, he created “End Result Cards” for his patients which included all diagnosis, procedures, and treatment for every one of his patients that he tracked for at least one year. He was also the first physician at Massachusetts General Hospital to institute a morbidity and mortality conference. Unfortunately, he lost his surgical privileges when he wanted to institute a plan for evaluating surgical competence. He went on to found his own hospital based on end-results and published these findings to the general public in 1916. He established the first bone tumor registry in the US and helped lead the founding of the American College of Surgeons and its Hospital Standardized Program, which eventually became the Joint Commission on Accreditation of Healthcare Organizations.

References

  1. Firkin BG and Whitwirth JA.  Dictionary of Medical Eponyms. 2nd ed.  New York, NY; Parthenon Publishing Group. 1996.
  2. Bartolucci S, Forbis P.  Stedman’s Medical Eponyms.  2nd ed.  Baltimore, MD; LWW.  2005.
  3. Yee AJ, Pfiffner P. (2012).  Medical Eponyms (Version 1.4.2) [Mobile Application Software].  Retrieved http://itunes.apple.com.
  4. Whonamedit – dictionary of medical eponyms. http://www.whonamedit.com
  5. Up To Date. www.uptodate.com
  6. A Study in Hospital Efficiency. Boston : Privately printed, 1916.
  7. Bone Sarcoma, an Interpretation of the Nomenclature Used by the Committee of the Registry of Bone Sarcoma of the American College of Surgeons. New York : P. B. Hoeber, 1925.

PAINE #PANCE Pearl – Hematology

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Question

You get a page from a gastroenterologist asking for a consult for platelet transfusion prior to an elective colonoscopy scheduled for tomorrow. The patient is a 56yo male, with a history of ITP and HTN. This procedure is for screening purposes only and the patient has not had any melena, hematochezia, or rectal bleeding. Platelet count is 97 x 109 per liter and the rest of the CBC is within normal limits.

  1. Does this patient meet the platelet transfusion threshold?
    1. If so, how much would you transfuse?
  2. If you were worried about bleeding, what are the other tests you could order to assess platelet function?

Answer

  1. The short answer is no, as he is not actively bleeding nor has any signs of anemia from chronic blood loss. Guidelines for platelet transfusions in preparation of invasive procedures is as follows:
    • < 100,000/microL for neurosurgery or ocular surgery
    • < 50,000/microL for most major surgery
    • < 50,000/microL for therapeutic endoscopic procedures
    • < 20,000/microL for low risk diagnostic endoscopic procedures
    • < 30,000/microL for bronchoscopy
    • < 20,000/microL for central line placement
    • < 20,000/microL for lumbar puncture with hematologic malignancy
    • < 50,000/microL for lumbar puncture without hematologic malignancy
    • < 80,000/microL for epidural anesthesia
    • < 20,000/microL for bone marrow biopsy
  2. The best way to assess platelet function is from a platelet function assay. This is generally determined by your facility’s lab, so it would be a good idea to talk with your lab to see what they use and how to interpret.

References

  1. Kumar A, Mhaskar R, Grossman BJ, et al. Platelet transfusion: a systematic review of the clinical evidence. Transfusion. 2015; 55(5):1116-27; quiz 1115. [pubmed]
  2. Warner MA, Woodrum D, Hanson A, Schroeder DR, Wilson G, Kor DJ. Preprocedural platelet transfusion for patients with thrombocytopenia undergoing interventional radiology procedures is not associated with reduced bleeding complications. Transfusion. 2017; 57(4):890-898. [pubmed]

Ep-PAINE-nym

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Virchow’s Triad

Other Known Aliases – none

Definition – triad of broad categories of factors that contribute to thrombosis: hypercoaguability, endothelial injury, and stasis of blood flow

Clinical Significance – These factors should always be considered in patients with suspected DVT, PTE, or acute arterial occlusion. Thought broad, they represent a simplistic mindmap to think of differential diagnoses and causes for patients with suspected conditions.

History – Names after Rudolf Ludwig Carl Virchow (1821-1902), who was a German physician and received his medical doctorate from the Friedrich-Wilhelms Institute in 1843. He had an interesting career in that he was a prolific writer (producing more than 2000 scientific manuscript), but also very politically charged and challenged not only the government, but also the status quo of medical education and dogmatism. This fervor allowed him to push the boundaries of what was known and being taught in medical schools and made him a well-known teacher, orator, and leader in the field of pathology. He first published his treatise on thrombosis in 1856 where he described his triad, but the eponym was not attributed to him until the mid-1900s.

References

  1. Firkin BG and Whitwirth JA.  Dictionary of Medical Eponyms. 2nd ed.  New York, NY; Parthenon Publishing Group. 1996.
  2. Bartolucci S, Forbis P.  Stedman’s Medical Eponyms.  2nd ed.  Baltimore, MD; LWW.  2005.
  3. Yee AJ, Pfiffner P. (2012).  Medical Eponyms (Version 1.4.2) [Mobile Application Software].  Retrieved http://itunes.apple.com.
  4. Whonamedit – dictionary of medical eponyms. http://www.whonamedit.com
  5. Up To Date. www.uptodate.com
  6. Virchow RLC. Gesammelte Abhandlungen zur wissenschaftlichen Medicin. Frankfurt am Main, 1 Meidinger, 1856+
  7. Bagot CN, Arya R. Virchow and his triad: a question of attribution. British journal of haematology. 2008; 143(2):180-90. [pubmed]

PAINE #PANCE Pearl – Hematology

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Question

You get a page from a gastroenterologist asking for a consult for platelet transfusion prior to an elective colonoscopy scheduled for tomorrow. The patient is a 56yo male, with a history of ITP and HTN. This procedure is for screening purposes only and the patient has not had any melena, hematochezia, or rectal bleeding. Platelet count is 97 x 109 per liter and the rest of the CBC is within normal limits.

  1. Does this patient meet the platelet transfusion threshold?
    1. If so, how much would you transfuse?
  2. If you were worried about bleeding, what are the other tests you could order to assess platelet function?

Ep-PAINE-nym

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Döhle Bodies

Other Known Aliases – none

Definition – light, blue-gray intra-cytosplasmic structures composed of agglutinated ribosomes most commonly found on neutrophils

Clinical Significance – These inclusions are thought to be the remnants of the rough endoplasmic reticulum and represent defects in cell production and maturation during granulocytopoesis. As a result, Döhle bodies are seen in patients with infection, inflammation, and/or high physiologic stress, but may also be seen in pregnancy.

History – Named after Karl Gottfried Paul Döhle (1885-1928), who was a German pathologist and received his medical doctorate from the University of Kiel in 1882. He joined the faculty at his alma mater (where he would remain for his entire career) as an assistant to Arnold Ludwig Heller in 1883. He was an introvert by nature and rarely attended medical conferences and published very little of his work, but was well-renowned across his university. His work with Heller on describing syphilitic aortitis was groundbreaking and what eventually brought him contemporary fame in the field of histopathology. He published his findings on his eponymous cells in an article in 1892

References

  1. Firkin BG and Whitwirth JA.  Dictionary of Medical Eponyms. 2nd ed.  New York, NY; Parthenon Publishing Group. 1996.
  2. Bartolucci S, Forbis P.  Stedman’s Medical Eponyms.  2nd ed.  Baltimore, MD; LWW.  2005.
  3. Yee AJ, Pfiffner P. (2012).  Medical Eponyms (Version 1.4.2) [Mobile Application Software].  Retrieved http://itunes.apple.com.
  4. Whonamedit – dictionary of medical eponyms. http://www.whonamedit.com
  5. Up To Date. www.uptodate.com
  6. Döhle KGP. Vorläufige Mittheilung über Blutbefunde bei Masern. Zentralblatt für allgemeine Pathologie und pathologische Anatomie. Jena. 1892;3:150-152.

PAINE #PANCE Pearl – Oncology

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Question

59yo male, with a history 27-pack-year history of smoking, presents to your primary care clinic for follow-up after a motor vehicle accident. He was evaluated in the local ED and was found to have a two masses on his liver on abdominal CT. What is the most likely cause of these lesions?

Answer

The most likely cause of this patient’s liver lesions is metastatic disease from distant primary malignancy. Hepatic metastases are 18-40 times more common than primary liver tumors due to the fact that liver is the primary filter for the body. In this particular patient, screening for lung cancer would be high on the list given his smoking history and a contrasted CT scan of the chest revealed a primary lung mass.

References

  1. Radiopaedia. Hepatic Metastases. https://radiopaedia.org/articles/hepatic-metastases-1
  2. Ananthakrishnan A, Gogineni V, Saeian K. Epidemiology of primary and secondary liver cancers. Seminars in interventional radiology. 2006; 23(1):47-63. [pubmed]

Ep-PAINE-nym

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Factor V Leiden

Other Known Aliases – rs6025

Definition – mutated form of factor V that is unable to bind to protein C and leads to a hypercoaguable state

Clinical Significance – This is the most common hereditary hypercoaguability disorder in patients with European lineage. It increases the lifetime risk of DV, PTE, and stroke and patient are managed with lifelong anticoagulation.

History – Named after the Dutch city of Leiden where it was first discovered by Professor Rogier Bertina and Professor Pieter Reitsma in 1994 and subsequently published in Nature in their article entitled “Mutation in blood coagulation factor V associated with resistance to activated protein C”. Leiden has been one of Europe’s most prominent scientific centres for more than 400 years. It contains the oldest university in the Netherlands and has produced 13 Nobel Prize winners.

References

  1. Firkin BG and Whitwirth JA.  Dictionary of Medical Eponyms. 2nd ed.  New York, NY; Parthenon Publishing Group. 1996.
  2. Bartolucci S, Forbis P.  Stedman’s Medical Eponyms.  2nd ed.  Baltimore, MD; LWW.  2005.
  3. Yee AJ, Pfiffner P. (2012).  Medical Eponyms (Version 1.4.2) [Mobile Application Software].  Retrieved http://itunes.apple.com.
  4. Whonamedit – dictionary of medical eponyms. http://www.whonamedit.com
  5. Up To Date. www.uptodate.com
  6. Bertina RM, Koeleman BP, Koster T, et al. Mutation in blood coagulation factor V associated with resistance to activated protein C. Nature. 1994; 369(6475):64-7. [pubmed]