NYACP Board Review Question of the Week
Every other Tuesday, NYACP members are sent a Board Review Question from ACP's MKSAP 18 to test professional knowledge and help prepare for the exam. Participant totals and answer percentages are distributed on Thursday in IM Connected, the Chapter's eNewsletter, and are also published on this page.
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August 2, 2022
MKSAP 18 Hematology and Oncology
Stem:
A 22-year-old man is evaluated for a mass on the left side of his neck. He has not experienced fevers, night sweats, or weight loss. He takes no medications.
On physical examination, all vital signs are normal. A left supraclavicular lymph node is enlarged to 4 cm, and axillary lymphadenopathy is noted bilaterally. There is no hepatosplenomegaly.
A biopsy specimen of the supraclavicular lymph node reveals Hodgkin lymphoma, nodular sclerosis subtype.
Laboratory results, including complete blood count, biochemical profile, and serum lactate dehydrogenase, are normal.
CT scan and PET scan show mediastinal lymphadenopathy, with nodes up to 4 cm in size, and left supraclavicular and bilateral axillary lymphadenopathy. No infradiaphragmatic disease is observed.
Which of the following is the most appropriate initial management?
Responses Received from Members (1,087 Responses):
The Correct Answer is: B. Chemotherapy
Educational Objective
Treat classic Hodgkin lymphoma.
Critique
The most appropriate management for this young man with Hodgkin lymphoma is chemotherapy. Hodgkin lymphoma encompasses four classic histologic subtypes (nodular sclerosing, mixed cellularity, lymphocyte predominant, and lymphocyte depleted) and one nonclassic subtype (nodular lymphocyte-predominant subtype expressing the CD20 cell surface antigen). The presentation is consistent among subtypes and is characterized by palpable, firm lymph nodes and, in some patients, B symptoms (fever, night sweats, weight loss). Other physical examination findings include splenomegaly (30%) and hepatomegaly (5%). Hodgkin lymphoma is a highly curable disease, and the best outcomes are achieved with systemic therapy, sometimes in combination with radiation therapy. In the United States, doxorubicin, bleomycin, vinblastine, and dacarbazine is the most commonly used chemotherapy regimen for classic Hodgkin lymphoma. The need for radiation after chemotherapy for early-stage disease depends on the initial bulk of disease, the response to treatment, and patient and institutional preference.
Bone marrow biopsy, in the absence of unexplained blood abnormalities, is not indicated in the evaluation of patients with Hodgkin lymphoma. Staging no longer includes routine bone marrow biopsy, lymphangiography, or staging laparotomy and splenectomy. High-resolution CT, PET, and the routine use of systemic therapy in even early-stage disease have obviated the need for these staging modalities. Patients with extensive lymph node involvement above the diaphragm are now typically treated with chemotherapy; therefore, there is no need for staging laparotomy and splenectomy to determine the presence of microscopic disease below the diaphragm as the treatment would be no different. With this approach, the short-term morbidity of the staging surgery and long-term risk of overwhelming sepsis from asplenia can be avoided.
Radiation therapy may have a role in this patient's management; however, it is given after completion of chemotherapy for consolidation. Although radiation therapy alone may have a high cure rate for classic Hodgkin lymphoma, the cure rate is higher and toxicity is reduced when chemotherapy is used as the primary treatment modality.
Key Point
All patients with classic Hodgkin lymphoma, regardless of stage, receive chemotherapy, usually the doxorubicin, bleomycin, vinblastine, and dacarbazine regimen, obviating the need for staging laparotomy and splenectomy.
Bibliography
Ansell SM. Hodgkin lymphoma: diagnosis and treatment. Mayo Clin Proc. 2015;90:1574-83. [PMID: 26541251] doi:10.1016/j.mayocp.2015.07.005
Copyright 2018, American College of Physicians.
July 19, 2022
MKSAP 18 Endocrinology and Metabolism: Item 49
Stem:
A 65-year-old woman is admitted to the hospital for fatigue and weakness over the last 1 to 2 weeks. Medical history is significant for hypertension, type 2 diabetes mellitus, and rheumatoid arthritis. For the past 3 months, the patient's rheumatoid arthritis has been treated with methotrexate and prednisone. Because of inadequate control, etanercept was added 2 weeks ago. At that time, the patient decided to discontinue prednisone due to increased bruising of her skin. Current medications are methotrexate, etanercept, amlodipine, folic acid, metformin, and aspirin.
On physical examination, blood pressure is 110/68 mm Hg sitting and 90/64 mm Hg standing, and pulse rate is 102/min sitting and 110/min standing. Symmetrical synovial bogginess is noted in the metacarpophalangeal joints and wrists bilaterally.
Laboratory studies show an 8 AM cortisol level of 2 µg/dL (55.2 nmol/L).
Which of the following is the most appropriate management?
Responses Received from Members (1,040 Responses):
The Correct Answer is: A. Initiation of hydrocortisone
Educational Objective
Treat secondary adrenal insufficiency.
Critique
This patient has secondary adrenal insufficiency, and hydrocortisone is the most appropriate treatment. Oral, injectable (including joint injections), and occasionally even topical glucocorticoids are able to suppress adrenocorticotropic hormone (ACTH) secretion. Glucocorticoids prescribed at doses above physiologic replacement for longer than 3 weeks should be tapered when discontinued allowing recovery of the pituitary-adrenal axis; if therapy has lasted less than 3 weeks, no taper is required for pituitary-adrenal axis recovery. The diagnosis of adrenal insufficiency is based on demonstrating inappropriately low serum cortisol levels. Because most assays measure total cortisol, abnormalities in cortisol-binding protein or albumin can trigger spurious results. An early morning (8 AM) serum cortisol of less than 3 μg/dL (82.8 nmol/L) is consistent with cortisol deficiency, whereas values greater than 15 to 18 μg/dL (414.0-496.8 nmol/L) exclude the diagnosis when binding protein abnormalities and synthetic glucocorticoid exposure are excluded.
Fludrocortisone in addition to hydrocortisone is unwarranted as fludrocortisone is needed only in primary adrenal insufficiency. There is no mineralocorticoid deficiency in secondary adrenal insufficiency.
An ACTH stimulation test is not necessary in this patient since the cortisol level less than 3 µg/dL (82.8 nmol/L) is diagnostic of adrenal insufficiency. If an ACTH stimulation test were necessary, dexamethasone can be given prior to the ACTH stimulation test since dexamethasone is not measureable in the cortisol assay. That is unnecessary given this patient's cortisol level.
Key Point
Oral, injectable (including joint injections), and even topical glucocorticoids are able to suppress adrenocorticotropic hormone (ACTH) secretion and result in secondary adrenal insufficiency.
Bibliography
Pazderska A, Pearce SH. Adrenal insufficiency - recognition and management. Clin Med (Lond). 2017;17:258-262. [PMID: 28572228] doi:0.7861/clinmedicine.17-3-258
Copyright 2018, American College of Physicians.