Student, Resident AND Fellow Research

Impressive work Physicians-in-training show their skills This year’s call for abstracts SC disease highlighting research and clinical work by Minnesota medical complicated by fat students, residents and fellows brought a smaller than usual syndrome response, but our reviewers were BY NOLAN ANDERSON AND SAMUEL T. IVES, MD impressed by the quality of the work. Introduction On exam, she was intubated and se- The reviewers looked at each emoglobin SC Disease is a variant of dated. She had “roving” eye movements manuscript to determine whether sickle cell disease (SCD), defined by but was not following commands, even off the research or case description Hthe presence of one allele each of HbS sedation. Lungs were clear. No petechiae was clear and complete, and HbC, which generally carries a better were seen. Labs were notable for elevated whether the methodology was than Hemoglobin SS Disease. transaminases, markedly elevated LDH, sound, whether the scientific Compared with Hemoglobin SS Disease, and worsening , drop- patients with Hemoglobin SC Disease ping from 160,000 per mm3 on admission literature review was sufficient 3 typically have fewer pain crises and longer to 50,000 per mm . A blood smear did and whether the findings had life expectancies.1 However, Fat Embolism show a leukoerythroblastic reaction. CT implications for future research. Syndrome (FES) is a rare devastating com- showed bilateral infiltrates concerning for The reviewer comments were plication of SCD that is more frequent in acute chest syndrome (Figure 1) and trans- 2,3 sent to those who had submitted patients with non-SS disease. We present thoracic echocardiogram showed severe abstracts, so they could respond. a case of FES leading to both respiratory pulmonary hypertension with acute cor failure and severe None of the reviewers called FIGURE 1 cerebral fat embo- CT scan showing bilateral infiltrates for major changes in any lism. manuscript. In fact, they said they thought the work was quite Case description good overall. A 26-year-old We thank our reviewers: woman with Hemo- Devon Callahan, MD; Siu-Hin globin SC Disease on hydroxyurea Wan, MD; and Barbara Yawn, was admitted for a MD. All are members of the vaso-occlusive pain Minnesota Medicine Advisory episode and initially Board treated with IV flu- ids, oxygen, and IV opiates. On hospital day 2, over several hours, she developed increasing hypoxia and obtundation, re- quiring ICU transfer and intubation.

38 | MINNESOTA MEDICINE | JULY/AUGUST 2018 Student, Resident AND Fellow Research pulmonale, but normal ejection fraction. appear within a few FIGURE 2 A bubble study demonstrated right-to- hours of presenta- TTE showing RV dilation left shunting associated with pulmonary tion, as in this case. hypertension. Due to her obtundation, an In a large review, MRI was done (Figure 3) which showed worsening anemia multiple diffuse acute infarcts and micro- or thrombocytope- hemorrhages consistent with acute infarc- nia was present in tions. the majority of pa- With the combination of exam find- tients.2,3 In a smaller ings (obtundation, hypoxia), lab findings group of patients, (thrombocytopenia, transaminase eleva- transaminase eleva- tion, and blood smear) and imaging (lung tion or renal failure infiltrates and diffuse ), she was was also seen. diagnosed with presumptive FES. Due to Diagnosis re- the risk of lung biopsy, she was treated for quires high clinical FES despite a confirmed biopsy. suspicion during She was treated with red cell exchange an acute episode. transfusion and discharged two weeks While bronchioal- after admission to a long-term acute-care veolar lavage or hospital. Five months after admission, marrow biopsy FIGURE 3 she had a fair neurologic recovery but re- could support the diagnosis, many cases mained unable to function independently. are confirmed with blood smear findings MRI showing diffuse cerebral infarcts compatible with bone marrow dysfunc- Discussion tion (e.g. leucoerythroblastic reaction) and FES is defined as histologically-proven imaging. organ involvement of fat embolism or Treatment of FES includes exchange respiratory failure and compatible neu- transfusion and supportive care. Patients rologic findings in a patient with known with FES are at very high risk of death and bone marrow necrosis.4 The hypothesis permanent neurologic impairment. is that either trauma or al- This case illustrates an extremely rare lows fat cells to be released from the bone but severe of SCD. Provid- marrow into circulation. Embolism to the ers must be aware of the risk of FES, par- lungs can cause severe acute pulmonary ticularly in patients with non-SS disease hypertension, predisposing to paradoxical presenting with vaso-occlusive pain crisis embolism in patients with a patent fora- complicated by respiratory distress or new men ovale or atrial septal defect. neurologic findings.

FES is seen most commonly in trauma Nolan Anderson is a fourth-year medical patients, but has been well described in student at the University of Minnesota Medical patients with SCD.5 Among SCD pa- School. Samuel T. Ives, MD, is an assistant professor at the University of Minnesota tients, FES is more common in women Medical School and practices in the division and among non-SS variants.2 It has been of General Internal Medicine, Hennepin Healthcare. bers. JAMA. 2014;312(10): 1033-48. described as precipitated by pregnancy or 4. Kosova E, Bergmark B, Piazza G. Fat embolism syn- parvovirus B19 infection.2,5 drome. Circulation. 2015. 131(3): 317-20. REFERENCES 5. Dang NC, Johnson C, Eslami-Farsani M, Haywood Clinical presentation is marked by re- LJ. Bone marrow embolism in sickle cell disease: a review. Am J Hematol. 2005. 79(1): 61-7. spiratory distress and neurologic findings 1. Platt OS, Brambilla DJ, Rosse WF et al. Mortality in (such as lethargy, confusion, agitation, sickle cell disease. Life expectancy and risk factors for early death. N Engl J Med. 1994:330(23): 1639-44. or ) in the setting of an acute vaso- 2. Tsiskikas DA, Gallinella G, Patel S, et al. Bone mar- occlusive pain episode. Symptoms often row necrosis and fat embolism syndrome in sickle cell disease: increased susceptibility of patients with non- SS genotypes and a possible association with human parvovirus B19 infection. Blood Rev. 2014;28(1). 23-30. 3. Yawn BP, Buchanan GR, Afenyi-Anna AN et al. Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel mem-

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