A rare case of follicular lymphoma transformed to a high-grade B-cell lymphoma in orbit

Transformation of lymphoma is an infrequent phenomenon, and involvement of the eye as such is even uncommon. Histological transformation in patients with follicular lymphoma who were previously treated with immune-chemotherapy carry a poor outcome. Here, we illustrate such a case with aggressive histological transformation from a low-grade lymphoma

A Snowball Effect; From an Uncomplicated Delivery to a Rare Case of Septic Ovarian Vein Thrombophelbitis Complicated by Ureteral Obstruction and Septic Pulmonary Emboli

Learning Objective #1: Recognize septic ovarian vein thrombophelbitis SVOT as an important cause of abdominal pain in peripartum females. Learning Objective #2: Know potential complications of SVOT that may occur beyond the pelvis. CASE: A 23 years old female, G2P2 presented 11 days post normal vaginal delivery with acute onset severe lower abdominal pain and substernal chest pain. She denied any change in the postpartum vaginal discharge and physical exam was only remarkable for diffuse abdominal tenderness. Computed Tomography CT of the abdomen/Pelvis and Chest were performed. CT abdomen revealed a thrombus in the inferior vena cava extending to the right adnexa suggestive of ovarian vein thrombus, and suspicious for thrombophlebitis, in addition to severe right hydroureter secondary to external obstruction. CT chest revealed lobar segmental pulmonary embolus as well as multiple patchy nodules suspicious for septic emboli. She was initially started on Ampicillin + Sulbactam, covering against common endometritis pathogens including streptococci, gram negatives and anaerobes, despite no growth of the blood cultures. She was also anti-coagulated with unfractionated heparin. She underwent placement of percutaneous nephrostomy tube for the un-resolving unilateral hydroureter. Subsequent workup for thrombophilia was negative and the patient was discharged home on warfarin, Clindamycin and Ceftriaxone for total 6 weeks. The percutaneous nephrostomy tube was removed 8 weeks later. The patient achieved a full recovery. IMPACT: This case of complicated septic ovarian vein thrombophelbitis SVOT made me realize that high index of suspicion of the diagnosis in peripartum patients with abdominal pain. Thoughtful consideration is needed to obtain the appropriate imaging modality (CT, US with dopplers or MRI of the abdomen), as well as the appropriate anatomical site imaged (lungs or the urinary system) to diagnose complications. DISCUSSION: Septic Ovarian vein thrombophlebitis SOVT complicated by septic pulmonary emboli and ureteral obstruction is very rare with few cases reported in the literature. SOVT mostly happens in the first 10 days postpartum with an incidence of 1 in 9000 of vaginal deliveries and 1 in 800 of caesarian deliveries. Other rare causes of SOVT include pelvic inflammatory disease and endometritis, malignancy and following pelvic surgery. The etiology for developing SOVT in the peripartum is proposed to be the general hypercoagulable state of pregnancy in addition to the stasis of ovarian venous drainage postpartum. Few studies suggested prothrombotic predisposition in up to 50% of SOVT cases. The diagnosis of SOVT is achieved radiologically utilizing ultrasound with doppler, CT or MRI of the abdomen. The current management of SVOT include anticoagulation and antibiotic therapy. Complications of SOVT include extension into the renal veins and the inferior vena cava, pulmonary emboli and sepsis. Few cases of SOVT complicated by ureteral obstruction are reported

A snowball effect; From an uncomplicated delivery to a rare case of septic ovarian vein thrombophelbitis complicated by ureteral obstruction and septic pulmonary emboli.

Learning Objective #1: Recognize septic ovarian vein thrombophelbitis SVOTas animportant cause of abdominal pain in peripartum females. Learning Objective #2: Know potential complications of SVOT that may occur beyond the pelvis. CASE: A 23 years old female, G2P2 presented 11 days post normal vaginal delivery with acute onset severe lower abdominal pain and substernal chest pain. She denied any change in the postpartum vaginal discharge and physical exam was only remarkable for diffuse abdominal tenderness. Computed To-mography CTof the abdomen/Pelvis and Chest were performed. CTabdomen revealed a thrombus in the inferior vena cava extending to the right adnexa suggestive of ovarian vein thrombus, and suspicious for thrombophlebitis, in addition to severe right hydroureter secondaryto external obstruction. CTchest revealed lobar segmental pulmonary embolus as well as multiple patchy nodules suspicious for septic emboli. She was initially started on Ampicillin + Sulbactam, covering against common endometritis pathogens including streptococci, gram negatives and anaerobes, despite no growth of the blood cultures. She was also anti-coagulated with unfractionated heparin. She underwent placement of percutaneous nephrostomy tube for the un-resolving unilateral hydroureter. Subsequent workup for thrombophilia was negative and the patient was discharged home on warfarin, Clindamycin and Ceftriaxone for total 6 weeks. The percutaneous nephrostomy tube was removed 8 weeks later. The patient achived a full recovery. IMPACT: This case of complicated septic ovarian vein thrombophelbitis SVOT made me realize that high index of suspicion of the diagnosis in peripartum patients with abdominal pain. Thoughtful consideration is needed to obtain the appropriate imaging modality (CT, US with dopplers or MRI of the abdomen), as well as the appropriate anatomical site imaged (lungs or the urinary system) to diagnose complications. DISCUSSION: Septic Ovarian vein thrombophlebitis SOVT complicated by septic pulmonary emboli and ureteral obstruction is very rare with few cases reported in the literature. SOVT mostly happens in the first 10 days postpartum with an incidence of 1 in 9000 of vaginal deliveries and 1 in 800 of caesarian deliveries. Other rare causes of SOVT include pelvic inflammatory disease and endometritis, malignancy and following pelvic surgery. The etiology for developing SOVT in the peripartum is proposed to be the general hypercoagu-lable state of pregnancy in addition to the stasis of ovarian venous drainage postpartum. Few studies suggested prothrombotic predisposition in up to 50% of SOVT cases. The diagnosis of SOVT is achieved radiologically utilizing ultrasound with doppler, CTor MRI of the abdomen. The current management of SVOT include anticoagulation and antibiotic therapy. Complications of SOVT include extension into the renal veins and the inferior vena cava, pulmonary emboli and sepsis. Few cases of SOVT complicated by ureteral obstruction are reported

Pheochromocytoma presenting as takotsubo cardiomyopathy: A nearly missed diagnosis in the absence of hypertension.

Pheochromocytoma (PCC) is a rare neuroendocrine tumor with a heterogeneous presentation. Headaches, palpitations and diaphoresis occur in 60-80% of cases, yet 8% are asymptomatic (1). Hypertension is found in 80-90%, although 13% are normotensive (1). The frequency of cardiovascular complications in PCC is not well studied, but reports of Takotsubo cardiomyopathy (TC) have been increasing (2). We report a case of a normotensive female presenting with TC and cardiogenic shock who was diagnosed with PCC. The diagnosis was considered only after the incidental discovery of an adrenal tumor. A 66-year-old female presented to the emergency department with acute onset chest pain, vomiting and back pain. She was hypotensive and tachycardic. Electrocardiogram showed sinus tachycardia and new anterior ST segment elevations. Laboratory evaluation revealed markedly elevated troponin. She also had acute kidney injury, acute liver injury with elevated transaminases \u3e1000 IU/L (reference/L) and elevated lactate. She underwent emergent cardiac catheterization showing normal coronary arteries. Echocardiogram revealed reduced ejection fraction (EF) of 13% with severe hypokinesis of the anterior, lateral and inferior wall. Takotsubo cardiomyopathy was diagnosed although she did not report any acute physical or emotional stress. Inotropic support and afterload reduction led to clinical improvement. Cardiac imaging 6 days later demonstrated dramatic improvement of EF to 64%. An abdominal ultrasound performed on presentation to evaluate the abnormal liver function revealed a right upper quadrant mass of uncertain origin. This prompted abdominal computed tomography which showed an 8.5 x 8 x 7.5 cm heterogeneous adrenal mass. On further questioning, the patient reported a 3-year history of debilitating episodes of substernal pressure associated with belching, vomiting, and palpitations. Her symptoms had been diagnosed as gastroesophageal reflux disease. PCC had not been considered since she was always normotensive. Hormonal testing to evaluate this mass revealed elevated total plasma free metanephrines 20,878 pg/ml (/ml) with normetanephrine 17,279 pg/ml and metanephrines 3599 pg/ml. Catecholamine excess from PCC was considered to be the cause of her TC. Doxazosin was titrated over 3 weeks for adequate alpha blockade and propranolol was added prior to surgery to control reflex tachycardia. She underwent a successful open adrenalectomy. Pathology confirmed PCC. Pheochromocytoma is a rare disease with variable presentation. Takotsubo cardiomyopathy is an increasingly recognized, yet still uncommon, presentation of PCC. In our patient, the diagnosis of PCC was entertained only after an adrenal mass was incidentally found on imaging. We suggest evaluation for PCC in patients with TC even in the absence of hypertension, as a missed diagnosis can have fatal consequences

It is not always about lady windermere: Cavitating lung disease due to mycobacterium avium complex.

Introduction: Pulmonary Mycobacterium avium complex (MAC) infections are becoming more prevalent in the general population; they usually occur in older, white patients with underlying pulmonary disease. We present a case of smoldering MAC infection in an immunocompetent black woman with no known pulmonary disease. Case: A 58-year-old black woman presented with a 1-day history of hemoptysis. Prior to this, she reported being in her usual state of health and had an extensive negative review of systems: fever, shortness of breath, cough, chest pain, fatigue, or weight loss. Social history was negative for smoking, alcohol, and illicit substance use; there was no concern for occupational exposure to particulate matter. There were no known risk factors for tuberculosis; her tuberculin skin test the prior year was nonreactive. Physical examination was only remarkable for tachycardia (114 beats/minute); examination of her respiratory and lymphatic systems were unrevealing. Initial investigations showed microcytic anemia (10.2 gm/dL), leukocytosis (16.7 K/uL), normal creatinine, and normal urinalysis. Her chest radiograph was concerning for multifocal airspace disease in the right lung and contrast-enhanced chest computed tomography (Figure 1) showed multiple lung cavities in the right lung with concomitant intrathoracic lymph node enlargement. She was empirically started on antibiotics for community-acquired pneumonia and priority was placed on ruling out pulmonary tuberculosis. Her first sputum sample had many acid-fast bacilli; Mycobacterium tuberculosis was not detected via nucleic acid amplification test. Work-up for human immunodeficiency virus and fungal infection were negative. Due to the sputum sample results, the local health department was contacted and it was noted that the patient had a MAC infection 3 years ago. She did not meet the treatment threshold at that time. MAC was later identified in the current sputum samples. She was discharged on ethambutol and clarithromycin. Outpatient appointments with ophthalmology and infectious diseases were also arranged. Discussion: MAC lung disease can present with a chronic cough and a myriad of constitutional symptoms: fever, fatigue, night sweats, weight loss, etc. It can mimic tuberculosis clinically and radiographically. Pulmonary MAC infections are more common in those that have underlying lung disease or are immunocompromised; however, it can occur in seemingly healthy individuals. A high index of clinical suspicion is needed to pursue the correct investigations as initial microbiological and radiological results can be nonspecific or point towards Mycobacterium tuberculosis. Historically, a MAC infection in an elderly woman with no preexisting lung disease was known as Lady Windermere syndrome

A rare case of follicular lymphoma transformed to a high-grade B-cell lymphoma in orbit

Transformation of lymphoma is an infrequent phenomenon, and involvement of the eye as such is even uncommon. Histological transformation in patients with follicular lymphoma who were previously treated with immune-chemotherapy carry a poor outcome. Here, we illustrate such a case with aggressive histological transformation from a low-grade lymphoma

Evaluating the clinical utility of testing for autoimmune disorders in the setting of leukopenia/neutropenia in an ambulatory hematology clinic.

Introduction: Leukopenia (WBC count of \u3c3.8K/uL) and/or neutropenia (\u3c1.8K/uL, both institutional lab cutoffs), are commonly encountered by physicians on routine blood testing on otherwise healthy patients. This finding, whether incidental or not, often leads to hematology referrals. A broad work up may be pursued in an attempt to find a definitive diagnosis. We conducted this study to assess the utility of testing for anti-nuclear antibody (ANA) as well rheumatoid factor (RF) in patients with leukopenia/neutropenia and whether or not a positive serology led to a rheumatologic diagnosis. Methods: After obtaining institutional review board approval, we searched for outpatient hematology consults with a diagnosis of leukopenia and/or neutropenia using ICD-9 and ICD-10 diagnostic codes between 2005-2015 at Henry Ford Hospital. Data points included demographics and antibody serology. Patients were excluded if they had a known hematologic disorder, known rheumatologic disorder or were on active chemotherapy. A positive ANA or RF were considered if titers were \u3e1:80 or RF \u3e14 IU/mL, respectively as these are institutional cutoffs. Complete blood counts were reviewed for patients who were not excluded based on above criteria. Hematology and rheumatology notes were reviewed looking for symptoms suggestive of autoimmune conditions or if an alternative diagnosis was made. Results: A total of 561 patients were seen in our outpatient clinic during the 10 year interval with an associated diagnostic code for leukopenia/neutropenia. 199 were excluded due to incomplete data or inadequate follow up period, 35 patients for known malignancy, 14 for known rheumatologic disorder, and 122 for being on active chemotherapy. The remaining 191 patients were reviewed for data analysis. Patient demographics are summarized in table 1. A total of 116 patients (61%) were tested for ANA, of which 27 (23%) were positive. 22 of those 27 (81%) patients were referred to rheumatology, 6 of which were diagnosed with rheumatologic diseases (22%). RF was tested in 110 patients (58%), of which 15 were positive (14%) all were referred to rheumatology, and 5(42%) were diagnosed with rheumatologic diseases. Test results, referral rate and rheumatologic diagnosis are listed in table 2. The majority of patients who were diagnosed with rheumatologic disorders had associated findings suggestive of underlying disease (3 patients with systemic lupus erythematosus (SLE) had mild thrombocytopenia (100-150K/uL), 1 patient with mixed connective tissue disease had Raynaud’s phenomenon, 3 patients with rheumatoid arthritis (RA) had arthralgias and 1 had myalgias, all with correlating physical exam or radiographic findings). Other alternative diagnoses for leukopenia/neutropenia included drug induced, viral and bacterial infections, hematologic malignancies, ethnic neutropenia, transient leukopenia, and idiopathic. Conclusion: Ambulatory hematology consults for leukopenia/neutropenia are a common occurrence in practice. Our study showed that 11 patients out of 191 (6%) were found to have an associated rheumatologic disorder, with the majority having other signs/symptoms or associated lab abnormalities suggestive of autoimmune disease. This study shows that testing for ANA and RF in patients with leukopenia/neutropenia are of limited clinical utility in asymptomatic patients with no other lab abnormalities and it should not be part of a routine work up unless directed by symptoms. Primary care providers need to screen for other abnormalities (symptoms/clinical findings) in order to identify patients that would benefit most from subspecialty referral

Evaluating the clinical utility of testing for autoimmune disorders in the setting of leukopenia/neutropenia in an ambulatory hematology clinic.

Introduction: Leukopenia (WBC count of \u3c3.8K/uL) and/or neutropenia (\u3c1.8K/uL, both institutional lab cutoffs), are commonly encountered by physicians on routine blood testing on otherwise healthy patients. This finding, whether incidental or not, often leads to hematology referrals. A broad work up may be pursued in an attempt to find a definitive diagnosis. We conducted this study to assess the utility of testing for anti-nuclear antibody (ANA) as well rheumatoid factor (RF) in patients with leukopenia/neutropenia and whether or not a positive serology led to a rheumatologic diagnosis. Methods: After obtaining institutional review board approval, we searched for outpatient hematology consults with a diagnosis of leukopenia and/or neutropenia using ICD-9 and ICD-10 diagnostic codes between 2005-2015 at Henry Ford Hospital. Data points included demographics and antibody serology. Patients were excluded if they had a known hematologic disorder, known rheumatologic disorder or were on active chemotherapy. A positive ANA or RF were considered if titers were \u3e1:80 or RF \u3e14 IU/mL, respectively as these are institutional cutoffs. Complete blood counts were reviewed for patients who were not excluded based on above criteria. Hematology and rheumatology notes were reviewed looking for symptoms suggestive of autoimmune conditions or if an alternative diagnosis was made. Results: A total of 561 patients were seen in our outpatient clinic during the 10 year interval with an associated diagnostic code for leukopenia/neutropenia. 199 were excluded due to incomplete data or inadequate follow up period, 35 patients for known malignancy, 14 for known rheumatologic disorder, and 122 for being on active chemotherapy. The remaining 191 patients were reviewed for data analysis. Patient demographics are summarized in table 1. A total of 116 patients (61%) were tested for ANA, of which 27 (23%) were positive. 22 of those 27 (81%) patients were referred to rheumatology, 6 of which were diagnosed with rheumatologic diseases (22%). RF was tested in 110 patients (58%), of which 15 were positive (14%) all were referred to rheumatology, and 5(42%) were diagnosed with rheumatologic diseases. Test results, referral rate and rheumatologic diagnosis are listed in table 2. The majority of patients who were diagnosed with rheumatologic disorders had associated findings suggestive of underlying disease (3 patients with systemic lupus erythematosus (SLE) had mild thrombocytopenia (100-150K/uL), 1 patient with mixed connective tissue disease had Raynaud’s phenomenon, 3 patients with rheumatoid arthritis (RA) had arthralgias and 1 had myalgias, all with correlating physical exam or radiographic findings). Other alternative diagnoses for leukopenia/neutropenia included drug induced, viral and bacterial infections, hematologic malignancies, ethnic neutropenia, transient leukopenia, and idiopathic. Conclusion: Ambulatory hematology consults for leukopenia/neutropenia are a common occurrence in practice. Our study showed that 11 patients out of 191 (6%) were found to have an associated rheumatologic disorder, with the majority having other signs/symptoms or associated lab abnormalities suggestive of autoimmune disease. This study shows that testing for ANA and RF in patients with leukopenia/neutropenia are of limited clinical utility in asymptomatic patients with no other lab abnormalities and it should not be part of a routine work up unless directed by symptoms. Primary care providers need to screen for other abnormalities (symptoms/clinical findings) in order to identify patients that would benefit most from subspecialty referral

Pheochromocytoma-Induced Takotsubo Cardiomyopathy

Pheochromocytoma, a rare catecholamine-secreting tumor, typically manifests itself with paroxysmal hypertension, tachycardia, headache, and diaphoresis. Less often, symptoms related to substantial hemodynamic compromise and cardiogenic shock occur. We report the case of a 66-year-old woman who presented with abdominal pain. Examination revealed a large right adrenal mass, cardiogenic shock, and severe heart failure in the presence of normal coronary arteries. Within days, the patient\u27s hemodynamic status and left ventricular ejection fraction improved markedly. Results of imaging and biochemical tests confirmed the diagnosis of pheochromocytoma-induced takotsubo cardiomyopathy. Medical therapy and right adrenalectomy resolved the patient\u27s heart failure, and she was asymptomatic postoperatively. We recommend awareness of the link between pheochromocytoma and takotsubo cardiomyopathy, and we discuss relevant diagnostic and management principles

Randomized controlled trial of convalescent plasma therapy against standard therapy in patients with severe COVID-19 disease

Convalescent plasma (CP) therapy in COVID-19 disease may improve clinical outcome in severe disease. This pilot study was undertaken to inform feasibility and safety of further definitive studies. This was a prospective, interventional and randomized open label pilot trial in patients with severe COVID-19. Twenty COVID-19 patients received two 200 ml transfusions of convalescent patient CP over 24-h compared with 20 who received standard of care. The primary outcome was the requirement for ventilation (non-invasive or mechanical ventilation). The secondary outcomes were biochemical parameters and mortality at 28 days. The CP group were a higher risk group with higher ferritin levels (p < 0.05) though respiratory indices did not differ. The primary outcome measure was required in 6 controls and 4 patients on CP (risk ratio 0.67, 95% CI 0.22–2.0, p = 0.72); mean time on ventilation (NIV or MV) did not differ. There were no differences in secondary measures at the end of the study. Two patients died in the control and one patient in the CP arm. There were no significant differences in the primary or secondary outcome measures between CP and standard therapy, although a larger definitive study is needed for confirmation. However, the study did show that CP therapy appears to be safe in hospitalized COVID-19 patients with hypoxia. Clinical trials registration NCT04356534: 22/04/2020