Acquired thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy with varied etiology and manifestations. It is uncommon for acute pancreatitis to trigger TTP. A 59-year-old man hospitalized with acute pancreatitis developed fever, acute kidney injury, thrombocytopenia, and microangiopathic hemolytic anemia (MAHA) on his second day in the hospital. Based on clinical suspicion and a high PLASMIC score indicating a severe deficiency in ADAMTS13 (a disintegrin-like and metalloprotease with thrombospondin type 1 motif no. 13) activity, a presumptive diagnosis of TTP was made. He was treated with plasmapheresis with improvement in his hemoglobin and platelet count. Severely deficient ADAMTS13 activity causing accumulation of large von Willebrand factor (VWF) multimers and subsequent formation of platelet rich microthrombi are thought to be the mechanisms of development of TTP. Proinflammatory mediators released during the systemic inflammatory response seen in acute pancreatitis can promote VWF activity and inhibit ADAMTS13 activity. Diffuse endothelial injury as a result of the inhibition of vascular endothelial growth factor (VEGF)mediated endothelial protection as well as production of excessive reactive oxygen species during an episode of acute pancreatitis also contributes to the pathogenesis of TTP. Thrombocytopenia and MAHA in a systemic inflammatory state should raise the suspicion for TTP. The PLASMIC score can further aid in the diagnosis and early initiation of plasmapheresis, which is key to the outcome.
ACE inhibitors are widely used and well-tolerated drugs. Angioedema is a well-known adverse effect, which involves the viscera rarely. This is a case of a 44-year-old African-American man with newly diagnosed hypertension, who presented with lower abdominal pain and diarrhoea. Based on the clinical picture and radiographic findings, lisinopril-induced intestinal angioedema was diagnosed. He recovered with supportive treatment, and the lisinopril was permanently discontinued. The mechanism of angioedema is thought to be the inhibition of ACE-mediated degradation of bradykinin, which is a peptide responsible for vasodilation and increased vascular permeability. While the external angioedema is unmistakable, intestinal angioedema has a relatively non-specific presentation and chronology, often leading to missed diagnosis and unnecessary interventions. Most common symptoms are abdominal pain and diarrhoea. Characteristic radiographic findings include ‘doughnut sign’ and ‘stacked coin’ appearance. Treatment is supportive. ACE inhibitors should be discontinued to prevent a recurrence.
While lung metastasis is common in renal cell carcinoma (RCC), isolated pleural involvement in rare, with only a handful of cases reported[1,2]. This is one such case, only the second case to be reported from the United States. CASE PRESENTATION: A 65-year-old female with coronary artery disease was evaluated in the emergency room with left sided chest pain, shortness of breath and non-productive cough for one week. The pain was non-radiating with no aggravating or relieving factors. She denied fever, chills, palpitations, or sick contacts. She endorsed a 50 pack-year smoking history. She was afebrile, tachycardic, hypertensive and hypoxic upon initial evaluation. Pertinent findings on physical examination include moderate respiratory distress with markedly diminished breath sounds on the left side. A complete blood count revealed neutrophilic leukocytosis (16x10^9/liter). Electrocardiogram and cardiac enzymes were unremarkable. A chest radiograph was notable for a large left sided pleural effusion. A computed tomography (CT) scan of chest demonstrated left pleural effusion, left lung atelectasis, multiple stable pulmonary nodules and an 8 x 8.5 centimeters (cm) lesion with combined solid and cystic elements in the upper pole of right kidney on the last few axial slices. She was treated with supplemental oxygen and empiric antibiotics for suspected bacterial pneumonia. A thoracentesis yielded 1500 milliliters of serosanguinous fluid. Analysis of the pleural fluid revealed papillary clusters of atypical cells with calcification. Upon immunohistochemical staining, these cells were found to be positive for vimentin, cluster of differentiation (CD) 10 and von Hippel Lindau (vHL) gene product. A positron emission tomography scan identified a thick-walled cystic lesion arising from the right kidney as a possible primary lesion. It also revealed multifocal pleural metastases as well as metastatic adenopathy of mediastinum and upper retroperitoneum. A diagnosis of stage IV renal cell carcinoma (Tx, N1, M1) made. She was started on pembrolizumab immunotherapy and is currently on it. DISCUSSION: RCC can present in a number of ways. Classic triad of RCC (flank pain, hematuria and palpable abdominal mass) is rare and usually indicates advanced stages of the disease. Most common metastatic sites include lungs, lymph nodes, bone, liver, and brain. Pulmonary involvement is commonly in the form of parenchymal metastasis. Pleural metastasis is usually secondary to the parenchymal involvement. A study of RCC in 1,451 autopsy cases demonstrated lung parenchymal metastasis in 76% cases and pleural metastasis in 12% cases[3]. However, none of them had isolated pleural metastasis. Isolated pleural involvement is rarely reported. Among such cases, the majority had a pleural tumor in association with the effusion. CONCLUSIONS: This is a case of RCC presenting initially as pleural effusion, without lung metastasis.
With more women getting pregnant at later ages than in the past, the incidence of malignancies in pregnancy is on the rise. Common malignancies of pregnancy are melanoma, breast cancer, cervical cancer, lymphomas, and leukemias. Colorectal carcinoma is rare in pregnancy, with an estimated incidence of 1 in 13',000 cases. We describe such a case of colorectal carcinoma in pregnancy (CRC-p), in a 31-year-old patient. She presented in the 21 st week of her second pregnancy with constipation of two weeks duration despite appropriate medical management. This prompted further evaluation with abdominal imaging revealing partial small bowel obstruction of unclear etiology. She was treated surgically with subtotal colectomy with ileostomy. Pathologic evaluation revealed Stage III B: pT3N2a adenocarcinoma with mucinous features of the sigmoid colon with lymph node metastases. Adjuvant FOLFOX chemotherapy was started in the third trimester and was continued postpartum for a total of 12 cycles. She is doing well, and ileostomy reversal is being planned at the time of writing this. Advancing maternal age is a significant risk factor for CRC-p. Common presenting symptoms in CRC-p include bleeding per rectum, abdominal pain, vomiting, and constipation. The frequent occurrence of many of these symptoms, as well as risks and restrictions associated with diagnostic modalities such as computed tomography scan and colonoscopy during pregnancy, makes the diagnosis challenging. Colonoscopy, followed by pathology evaluation, remains the standard diagnostic method in CRC-p. Management of CRC-p is determined by multiple variables such as the stage of the disease, gestational age, and most importantly, patient wishes. Surgical resection is performed following the diagnosis if the gestational age is less than 20 weeks and delayed until after delivery if gestational age is above 20 weeks. 5fluorouracil based chemotherapy regimens are used in second and third trimesters, in patients with stage III CRC-p. Prognosis has been reported variably. Despite advanced stages at presentation, most of the studies indicate a similar prognosis compared to CRC in the nonpregnant population. Two-year survival was found to be 64.4% in one case series.
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