allergies: patient recorded as having no known allergies to drugs attending: chief complaint: fever, pancytopenia, ruq pain. major surgical or invasive procedure: bone marrow biopsy ercp with cbd stent placement central line/hd line placement intubation lumbar puncture bronchoscopy history of present illness: hpi: 46 yo m with h/ s/p thymectomy on imuran who initially presented to his pcp t103 and dry cough treated with amoxicillin and augmentin without improvement. he was then admitted to an osh on for pancytopenia (wbc 2.6, 18% bands, plt 104) and elevated lfts c/w cholestasis. he was treated with azythromycin and atovaquone for suspected tick borne illness. he had a positive monospot test. hepatitis serologies were negative. . patient was admitted to the surgical service at on for persisitent fever and an elevated direct tbili thought to be secondary to cholangitis. he was started on unasyn. he underwent an ercp on which did not show biliary tract obstruction, however, a cbd stent was placed. he was transfused 1 unit of prbc's, 3 bags of ffp, and 3 bags of plts. . prior to the ercp he developed repsiratory distress and was intubated. cxr revealed bilateral patchy pulmonary infiltrates. he became hemodynamically unstable and he was started on norepi gtt. id was consulted and ceftriaxone/ doxycyclin were added; zosyn was d/c'ed. he spiked a temp to 105.3. he was transfered to the micu on for further managament. past medical history: - myasthenia for 19 years s/p thymectomy - migraines - prednisone induced osteoporosis - low back pain social history: has a girlfriend. a 14 yo son who recently had a cold. lives with girlfirend and step children. smokes and drinks etoh occassionally. no hx of ivdu. lives in near a swamp. breakheart reservation is 2 miles away. no hx of tick bites. family history: mother has htn. physical exam: upon transfer to : tm 102.2 tc 97.6 bp 175/92 (108-175/52-92) hr 89 (71-111) ps 5/0 fio2 35% vt 850 (700-850) rr 16; abg 7.44/33/173/23 fentanyl 125; off midaz since gen: sedated/intubated, appears comfortable on ventilator, occasional hiccups heent: et tube in place, eyes with lubricant, perrl, pupils pinpoint cv: distant heart sounds. no murmurs appreciated. resp: anteriorly - crackles throughout abd: soft, distended, decreased bm, unable to appreciate hsm skin: warm. well perfused. ext: hyperreflexic, spastic, 5 beats of myoclonus, toes upgoing, strong dp/pt pulses access: right ij triple lumen placed , left ij temp dialysis cath placed by ir pertinent results: liver us : 1. marked gallbladder wall edema with an effaced, non-distended gallbladder lumen is noted, without intrahepatic biliary ductal dilatation. there is minimal pericholecystic fluid. 2. dilatation of the proximal cbd. 3. prominent periportal lymphadenopathy, nonspecific. . cxr : new perihilar pulmonary edema and bilateral pleural effusions. . ercp : ccannulation of the common bile duct. cholangiogram demonstrates a normal caliber of the common bile duct and intrahepatic ducts. the cystic duct is also filled with contrast, partially opacifying the gallbladder. no strictures or filling defects are identified. following cholangiogram, there is placement of a plastic stent within the common bile duct. . ct chest/abd/pelvis : 1. multifocal pulmonary opacities, which could represent an infectious process. 2. bilateral axillary and right hilar lymphadenopathy, all could be related to the underlying infectious process. 3. moderate bilateral pleural effusions. 4. although there is ascites, fluid within the lesser sac and adjacent to the pancreatic head raises the suspicion of pancreatitis. 5. splenomegaly. 6. periportal lymphadenopathy. . echo : no evidence of endocarditis. normal global and regional biventricular systolic function. mild mitral regurgitation. . immunophenotyping : pending . bronchial washings : negative for malignant cells. pulmonary macrophages. no viral cytopathic changes or microorganisms seen. . r leni : no evidence of right lower extremity dvt. . cxr : pulmonary edema, now mild, has improved substantially since . a relatively rapid onset between and 16 and pace of improvement suggests the diagnosis is cardiogenic rather than noncardiac edema. heart is normal size. there is no mediastinal or pulmonary vascular engorgement. lungs are clear aside from bands of atelectasis. other pleural surfaces are normal except for mild thickening associated with fractures of left ribs at least the fifth, which may have developed between and 21. tip of the right jugular line projects over the junction of the brachiocephalic veins and a left internal jugular line ends in the upper svc. . ct abd/pelvis : 1. slightly increased amount of intraabdominal simple free fluid. 2. interval placement of cbd stent with collapsed, edematous gallbladder. 3. pancreas appears similar to previous exam. . ct head : 1. no acute intracranial hemorrhage or mass effect. 2. interval opacification of multiple mastoid air cells. . 09:46pm blood hcv ab-negative 10:21pm blood hiv ab-negative 09:46pm blood hbsag-negative hbsab-negative igm hav-negative 06:30am blood tsh-2.9 10:21pm blood caltibc-156* vitb12-1420* folate-14.4 hapto-65 ferritn-6065* trf-120* 02:36am blood lipase-760* 06:25am blood lipase-242* 01:20pm blood alt-81* ast-240* alkphos-294* amylase-147* totbili-8.9* dirbili-7.4* indbili-1.5 11:35pm blood alt-90* ast-361* ld(ldh)-665* ck(cpk)-725* alkphos-217* amylase-203* totbili-7.5* 06:20am blood alt-85* ast-21 alkphos-136* totbili-1.7* 01:20pm blood urean-16 creat-1.0 na-131* k-4.1 cl-98 hco3-24 angap-13 06:30pm blood glucose-111* urean-88* creat-7.1* na-130* k-4.9 cl-98 hco3-18* angap-19 07:40am blood glucose-87 urean-30* creat-1.1 na-138 k-3.8 cl-105 hco3-25 angap-12 03:23am blood wbc-3.5* lymph-17* abs -595 cd3%-97 abs cd3-580 cd4%-89 abs cd4-532 cd8%-8.5 abs cd8-51* cd4/cd8-9.9* 07:40am blood gran ct-70* 01:20pm blood wbc-2.0* rbc-3.50* hgb-11.5* hct-33.5* mcv-96 mch-32.8* mchc-34.3 rdw-15.6* 07:40am blood wbc-0.4* rbc-2.72* hgb-8.1* hct-22.6* mcv-83 mch-29.9 mchc-35.9* rdw-14.7 plt ct-81*# 02:14pm cerebrospinal fluid (csf) wbc-2 rbc-0 polys-0 lymphs-87 monos-13 11:34pm cerebrospinal fluid (csf) wbc-13 rbc-3* polys-0 lymphs-95 monos-0 macroph-5 11:34pm cerebrospinal fluid (csf) wbc-13 rbc-32* polys-0 lymphs-67 monos-0 macroph-33 02:13pm cerebrospinal fluid (csf) totprot-34 glucose-61 11:34pm cerebrospinal fluid (csf) totprot-19 glucose-63 . bm bx erythroid-dominant marrow with increased hemophagocytic histiocytes, decreased cellular density, and increased background eosinophilic cell debris, consistent with hemophagocytic syndrome (hemophagocytic lymphohistiocytosis brief hospital course: hospital course: . # fever/pancytopenia/id/hemophagocytic lymphohistiocytosis: pt had been afebrile since and all abx d/c'ed on , however, vanco/ceftaz were restarted on for tm of 102.2. concern was for vap given increased respiratory secretions vs recurrent pancreatitis with pseudocyst as pancreas enzymes were rising after recently having restarted tf. ct abd/pelvis was without evidence of worsening radiographic pancreatitis. other sources considered included line infections (right ij recently replaced in same site) or c. diff given prolonged abx course. . extensive prior infectious workup had revealed a positive ebv igm, ebv pcr, and ebv pcr in csf. given pancyotpenia, splenomegaly, and ebv infection heme/onc and id were considered the diagnosis of hemophagocytic lymphohistiocytosis, which was confirmed by repeat bone marrow biopsy (first biopsy unremarkable). pt was begun on etoposode, ivig and decadron on ~. his pancytopenia was also treated with epogen and neupogen. . per heme, hlh likely triggered by underlying ebv infection. while there was evidence of ebv in the csf; because of normal protein no need for it-mtx. the patient was started on a steroid taper (currently on 10 mg decadron) and will need 8 weeks total of etoposide. renal failure, pancreatic abnormalities, and elevated lfts all thought to be d/t underlying hlh. in addition, id consults did not recommend treating ebv viremia with anti-virals. . on , pt was noted to have ebsl klebsiella in a sputum and bal sample, and was begun on meropenem for 14 day course. he continued to develop low grade temperature (100.0-100.6), which were attributed to his hlh, ivig, and cvvhd. . # respiratory failure: pt intubated on for impending respiratory distress at time of his ercp. upon admission to the on , his respiratory mechanics had improved considerably, and he was oxygenating and ventilating well on ps 5/0. initially unable to extubate secondary to altered mental status and increased secretions. pt was often desyncrhonous on vent secondary to hiccups when sedation weaned. as mental status improved gradually, pt was extubated on . his respiratory status continued to improve slowly, despite +bal for ebsl klebsiella and total body fluid overload, and on pt was sat'ing >95% on ra. . # mental status - pt presented to alert & oriented, however his mental status subsequently declined. after intubation on , pt remained largely sedated until just prior to admission to the on . attempts to wean sedation were limited by hiccups which resulted in dysynchrony the mechanical ventilation, breif neuro exam at time of admission with sedation weaned revealed pt responsive only to deep painful stimulus (sternal rub), pupils minimally reactive to light bilaterally, gag was present, with slow corneal reflex. +hyperreflexia, though tone was flacid, and 5-10 beat clonus of both feet was noted which initially worsened to 20 beat clonus on before slowly improving. . was seen by the neurology/psychiatry services given his new neurological findings and h/o myasthenia (which predominantly was ocular per pt's family). eeg was obtained which showed diffuse slowing, but no focus of seizure activity. ct head on unremarkable. over the course of his first week in the , pt's mental status improved dramatically, presumably with chemotherapy. by pt was alert, pleasantly conversive, and following all commands. his imuran for myasthenia has been held since admission. neuro also noted proximal weakness of his arms, which improved during his hospital course. per neruo, he should hold imuran until he follows up with neuro as an outpatient. . # renal - pt without h/o cri, developed arf likely secondary to atn from hypotension and underlying hlh on . pt was started on cvvhd at that time for volume overload anuria, however, uop gradually improved, and on pt was discontinued from hd. creatinine normal on discharge. . # cholestasis/hepatitis/pancreatitis - pt presented to from osh with ruq pain, fever, and elevated lfts (tbil 7's), for which he underwent ercp with cbd stent on . lfts have since trended down, though amylase/lipase (peak in 1000s) were starting to plateau at 500s on . ct abdomen showed pancreatic fluid collection, but not psuedocyst or necrosis. on pt denied abdominal pain, and was hungry, thus was transitioned from tpn to tf cautiously, as prior attempt to restart tube feeds was limited by bump in amylase/lipase. on , pt was tolerating tf without difficulty, in addition to sips of clear liquids, thus he was advanced to a regular diet after a speech & consult was obtained. on the floor, he tolerated his diet without other clinical s/sx of pancreatitis. . psych: thought the patient had a mild encephalopathy that was slowly resolving. recommeded haldol/seroquel for sleep; however, this made the patient feel strage. given resolution of ms changes, ok for patient to receive ambien at rehab prn. . htn: kept on lopressor 100 mg tid with excellent results. medications on admission: imuran, imitrex, amoxicillin, augmentin, atovaquone, azithromax discharge medications: 1. metoprolol tartrate 50 mg tablet sig: two (2) tablet po tid (3 times a day). disp:*180 tablet(s)* refills:*2* 2. meropenem 500 mg recon soln sig: five hundred (500) mg intravenous every eight (8) hours: please continue until anc >500. disp:*qs mg* refills:*2* 3. dexamethasone 4 mg tablet sig: 2.5 tablets po once a day: please give 10 mg po daily until ; then begin 5 mg po daily. disp:*qs tablet(s)* refills:*2* 4. acetaminophen 325 mg tablet sig: one (1) tablet po q4-6h (every 4 to 6 hours) as needed for fever. disp:*qs tablet(s)* refills:*0* 5. pantoprazole 40 mg tablet, delayed release (e.c.) sig: one (1) tablet, delayed release (e.c.) po once a day: while on steroids. disp:*30 tablet, delayed release (e.c.)(s)* refills:*2* 6. miconazole nitrate 2 % powder sig: one (1) appl topical tid (3 times a day) as needed. disp:*qs qs* refills:*0* 7. nystatin 100,000 unit/g cream sig: one (1) appl topical (2 times a day). disp:*qs qs* refills:*2* 8. ambien 5 mg tablet sig: one (1) tablet po at bedtime as needed for insomnia. disp:*30 tablet(s)* refills:*0* 9. fluconazole 200 mg tablet sig: two (2) tablet po once a day: please continue until anc >500. . disp:*60 tablet(s)* refills:*2* discharge disposition: extended care facility: & rehab center - discharge diagnosis: 1. hemophagocytic lymphohistiocytosis 2. acute renal failure, resolved 3. elevated lfts secondary to obstruction/hlh 4. elevated amylase/lipase, likely secondary to hlh 5. myasthenia , stable 6. hospital acquired pna (klebsiella) 7. pancytopenia/febrile neutropenia 8. sepsis 9. respiratory failure 10. hypertension discharge condition: stable discharge instructions: please contact dr. office or your pcp should you develop any fevers, chills, sweats, abodminal pain, nausea, vomiting, or any other complaints. please make an appointment to see your outpatient neurologist as soon as possible. followup instructions: provider: , md phone: date/time: 11:00 provider: , md phone: date/time: 11:00 someone from the gastroenterology team will be calling you at rehab regarding pulling the stent from your liver. please f/u with your neurologist as an outpt. Procedure: Venous catheterization, not elsewhere classified Continuous invasive mechanical ventilation for 96 consecutive hours or more Spinal tap Incision of lung Spinal tap Incision of lung Parenteral infusion of concentrated nutritional substances Insertion of endotracheal tube Enteral infusion of concentrated nutritional substances Hemodialysis Venous catheterization for renal dialysis Biopsy of bone marrow Biopsy of bone marrow Closed [endoscopic] biopsy of bronchus Closed [endoscopic] biopsy of bronchus Endoscopic insertion of stent (tube) into bile duct Transfusion of packed cells Transfusion of other serum Injection or infusion of cancer chemotherapeutic substance Transfusion of platelets Injection or infusion of immunoglobulin Infusion of vasopressor agent Diagnoses: Hypocalcemia Acute kidney failure with lesion of tubular necrosis Congestive heart failure, unspecified Toxic encephalopathy Unspecified essential hypertension Adrenal cortical steroids causing adverse effects in therapeutic use Severe sepsis Acute respiratory failure Other specified septicemias Septic shock Cholangitis Hepatitis, unspecified Acute pancreatitis Pneumonia due to Klebsiella pneumoniae Other osteoporosis Chronic ulcer of other specified sites Myasthenia gravis without (acute) exacerbation Hiccough Infectious mononucleosis Other specified disorders of metabolism