Teaching rounds with Dr Panella covered a case of head and neck cancer with an unusual neuroendocrine tumor located in the tonsillar fossa. Some key points of evaluating a patient with a neck mass were discussed. If the mass is tender and infection is a possibility, a two week trial of antibiotics is a reasonable first step. If the mass persists beyond that point, invasive testing with FNA is appropriate as well as consideration of CT imaging. This AAFP article has a nice review of the topic.
Another case discussed this week was a patient that was admitted with progressive pain and malaise and was found to have a markedly elevated WBC at her oupatient visit (>80K). Her peripheral smear supported a diagnosis of CLL but this alone did not completely explain her multifocal pain. A bone scan was requested that revealed multifocal osteoblastic disease. Bone marrow aspirate revealed metastatic adenocarcinoma, indicating the simultaneous presentation of 2 malignancies.
Pearls:
CLL is the most common type of leukemia in adults. Many patients are diagnosed incidentally when a CBC is checked for other reasons. Other cases are identified during evaluation of patients for unexplained lymphadenopathy. Very few cases are symptomatic, and when symptoms occur it usually consists of fevers, chills, and night sweats (B symptoms) and not pain. This disease is usually managed with watchful waiting, but new advances in prognostic markers may push some patients towards earlier treatment if they have high risk features. See this NEJM review for more details. Alemtuzumab is a monoclonal antibody therapy (anti CD52) approved for therapy of CLL.
CLL patients seem to be at risk of developing other malignancies, particularly other leukemias, lymphomas, melanomas and sarcomas. The fact that CLL is often asymptomatic makes it more likely that the CLL will not be discovered until workup for the other malignancy, making them appear to be "simultaneous", though the CLL may have been present for quite some time.
Good pictures from American Society of Hematology Atlas of CLL shown here.
Review of topics covered in morning report, M&m, and core conferences with links to supplemental reading material.
Subscribe
Posts
All Comments
Friday, May 28, 2010
Tuesday, May 25, 2010
MR 19 - 21 May
Pseudoseizures: Difficult and challenging condition for patients and physicians alike. Suspect this in patients with epilepsy that is refractory to antiepileptic drugs or in patients with significant emotional distress or personality disorders. Do not forget that up to 1/3 of patients that have pseudoseizures will also have true epilepsy, making management particularly difficult in these folks. Cornerstone of diagnosis is video ECG monitoring.
Monday, May 17, 2010
MR 17 May 2010
Impromptu teaching rounds for me this AM. Complex case involving a hispanic female with a pain flare in multiple joints, recent prolonged travel in Mexico complicated by diarrhea, and a history of unexplained iron deficiency anemia. Important turning point in this case was measurement of ESR of >115. XRAY of spine and pelvis revealed fused SI joints and enthesopathic changes of the vertebra consistent with ankylosing spondylitis vs enteropathic arthropathy.
Seronegative spondyloarthropathy: constellation of diseases that include ankylosing spondylitis, reactive arthritis (Reiter's), psoriatic arthritis, enteropathic arthritis, and undifferentiated spondys. These diseases are often associated with HLA-B27. Enteropathic arthritis can be associated with a variety of infections to include traveler's diarrhea and brucellosis. IBD associated spondy can preceed onset of bowel symptoms by months / years. Treatment of these disease ranges from anti-inflammatories, corticosteroids, to TNF alph inhibitors.
Seronegative spondyloarthropathy: constellation of diseases that include ankylosing spondylitis, reactive arthritis (Reiter's), psoriatic arthritis, enteropathic arthritis, and undifferentiated spondys. These diseases are often associated with HLA-B27. Enteropathic arthritis can be associated with a variety of infections to include traveler's diarrhea and brucellosis. IBD associated spondy can preceed onset of bowel symptoms by months / years. Treatment of these disease ranges from anti-inflammatories, corticosteroids, to TNF alph inhibitors.
Sunday, May 16, 2010
MR Potpourri Through May 14
No MR blog updates in recent weeks due to ACP and vacation. Had a few rare diseases recently - here are some recent pearls:
Neurofibromatosis: Autosomal dominant disease, but half of cases arise from spontaneous mutations. NF 1 has prominent cutaneous manifestations (fibromas, axillary freckling, cafe-au-lait spots). You can observe lisch nodules in the iris of affected individuals. NF2 has predominant central manifestations and less cutaneous disease. Think this dx in a patient with bilateral acoustic neuromas. Differentiate from tuberous sclerosis, another AD disease. Predominant facial lesions known as adenoma sebaceum, central nervous system tumors, renal tumors (angiomyolipomas), developmental delay, and seizures are prominent findings. Some cutaneous lesions likely to show up in challenge bowl include periungual fibromas and ash leaf macules.
Marfan Syndrome: multisystem disorder with prominent aortic pathology (aortic regurgitation). Primary defect is mutation in the fibrillin gene. Patients are usually tall with unusually long limbs and fingers for height. This disorder is a cause of sudden death in young athletes. Differentiate from Ehlers-Danlos syndrome - multiple genetic defects with Type V and III collagen being the most prominent. Skin and tendon laxity are prominent in this condition. Mitral valve prolapse and regurgitation is the most common cardiac manifestation. Osteogenesis imperfecta is caused by Type I collagen deficiency, with hallmark findings of fragile bones and blue sclerae. (Aside - the villain played by Samuel L Jackson in Unbreakable was an OI sufferer)
HIV Infection: Covered recently in a couple morning reports, but one relatively new development in the field: The new treatment initiation threshold is recommend at CD4 counts less than 500, and the guidelines indicate that treatment can be considered at any CD4 count on a case by case basis with highly motivated patients. The guidelines panel was split 50/50 regarding making this latter threshold a formal recommendation.
Narrative Medicine: Dr Lands' Grand Rounds on narrative medicine was one to remember. The stories we tell tie us together as a profession and give practice deeper meaning than simple facts, stats, and p values. A poem in the sudden death article linked above brings that home, and I close this entry with it:
The time you won your town the race / We chaired you through the market-place;
Man and boy stood cheering by, / And home we brought you shoulder-high.
To-day, the road all runners come, / Shoulder-high we bring you home,
And set you at your threshold down, / Townsman of a stiller town.
— A.E. Housman, "To an Athlete Dying Young" (1895)
Neurofibromatosis: Autosomal dominant disease, but half of cases arise from spontaneous mutations. NF 1 has prominent cutaneous manifestations (fibromas, axillary freckling, cafe-au-lait spots). You can observe lisch nodules in the iris of affected individuals. NF2 has predominant central manifestations and less cutaneous disease. Think this dx in a patient with bilateral acoustic neuromas. Differentiate from tuberous sclerosis, another AD disease. Predominant facial lesions known as adenoma sebaceum, central nervous system tumors, renal tumors (angiomyolipomas), developmental delay, and seizures are prominent findings. Some cutaneous lesions likely to show up in challenge bowl include periungual fibromas and ash leaf macules.
Marfan Syndrome: multisystem disorder with prominent aortic pathology (aortic regurgitation). Primary defect is mutation in the fibrillin gene. Patients are usually tall with unusually long limbs and fingers for height. This disorder is a cause of sudden death in young athletes. Differentiate from Ehlers-Danlos syndrome - multiple genetic defects with Type V and III collagen being the most prominent. Skin and tendon laxity are prominent in this condition. Mitral valve prolapse and regurgitation is the most common cardiac manifestation. Osteogenesis imperfecta is caused by Type I collagen deficiency, with hallmark findings of fragile bones and blue sclerae. (Aside - the villain played by Samuel L Jackson in Unbreakable was an OI sufferer)
HIV Infection: Covered recently in a couple morning reports, but one relatively new development in the field: The new treatment initiation threshold is recommend at CD4 counts less than 500, and the guidelines indicate that treatment can be considered at any CD4 count on a case by case basis with highly motivated patients. The guidelines panel was split 50/50 regarding making this latter threshold a formal recommendation.
Narrative Medicine: Dr Lands' Grand Rounds on narrative medicine was one to remember. The stories we tell tie us together as a profession and give practice deeper meaning than simple facts, stats, and p values. A poem in the sudden death article linked above brings that home, and I close this entry with it:
The time you won your town the race / We chaired you through the market-place;
Man and boy stood cheering by, / And home we brought you shoulder-high.
To-day, the road all runners come, / Shoulder-high we bring you home,
And set you at your threshold down, / Townsman of a stiller town.
— A.E. Housman, "To an Athlete Dying Young" (1895)
Subscribe to:
Posts (Atom)