Successful Treatment of Massive Pulmonary Embolism in Pregnancy with Catheter-Directed Embolectomy.

Introduction Pulmonary embolism (PE) is associated with approximately 10.5% of maternal deaths in the United States. Despite heightened awareness of its mortality potential, there islittle data available to guide its management in pregnancy. We present the case of a massive PE during gestation successfully treated with catheter-directed embolectomy. Case Presentation A 37-year-old G2P1001 presented with a syncopal episode preceded by dyspnea and chest pain. Upon presentation, she was hypotensive, tachycardiac, and hypoxic. Imaging showed an occlusive bilateral PE, right heart strain, and a possible intrauterine pregnancy. Beta-human chorionic gonadotropin was positive. She was taken emergently for catheter-directed embolectomy. Her condition immediately improved afterward. Postprocedure pelvic ultrasound confirmed a viable intrauterine pregnancy at 10 weeks gestation. She was discharged with therapeutic enoxaparin and gave birth to a healthy infant at 38 weeks gestation. Conclusion Despite being the gold standard for PE treatment in nonpregnant adults, systemic thrombolysis is relatively contraindicated in pregnancy due to concern for maternal or fetal hemorrhage. Surgical or catheter-based thrombectomies are rarely recommended. Limited alternative options force their consideration, particularly in a hemodynamically unstable patient. Catheter-directed embolectomy can possibly bypass such complications. Our case exemplifies the consideration of catheter-directed embolectomy as the initial treatment modality of a hemodynamically unstable gestational PE.

Pseudo-Thrombotic Microangiopathy Secondary to Vitamin B12 Deficiency.

Background: Clinical B12 deficiency with hematological or neurological manifestations is rare. An unusual manifestation of B12 deficiency is pseudo-thrombotic microangiopathy (TMA), which is characterized by hemolytic anemia, thrombocytopenia, and schistocytosis and only occurs in 2.5% of those with B12 deficiency. Pseudo-TMA is misdiagnosed as thrombotic thrombocytopenic purpura (TTP) in 40% of cases, resulting in misguided treatment including plasmapheresis. Case: A 44-year-old Hispanic presented with 3 weeks of progressively worsening non-radiating chest pain, fatigue, and shortness of breath (SOB). Laboratory findings revealed severe pancytopenia and macrocytosis with a hemoglobin of 5.4 g/dL, mean corpuscular volume of 116.3 fL, and vitamin B12 low at 149 pg/mL. She was diagnosed with pseudo-TMA and after starting 1000 micrograms of parenteral vitamin B12 injections daily and discontinuing plasmapheresis and steroid administration, she improved. Conclusion: Failure to recognize pseudo-TMA often results in unnecessary treatment with plasmapheresis and delays appropriate treatment with vitamin B12 supplementation. It is therefore extremely important to consider pseudo-TMA as a differential diagnosis in patients that present with hemolytic anemia, thrombocytopenia, and schistocytosis.

A 24-Year-Old Man Presenting with Lung Metastases from a Primary Retroperitoneal Extragonadal Choriocarcinoma.

BACKGROUND Primary retroperitoneal choriocarcinoma is a rare form of extragonadal germ cell tumor that is highly aggressive and responds poorly to chemoradiation. Extragonadal choriocarcinomas are notoriously challenging to diagnose, and have often progressed to advanced disease by the time of diagnosis. The survival rate for extragonadal choriocarcinoma is approximately 30%, which is much lower than that of extragonadal non-seminomatous germ cell tumors (GCT) in general. CASE REPORT A 24-year-old man with no significant past medical history presented with left-sided, pleuritic chest pain and back pain radiating down his left leg, of 1-year duration. Computed tomography (CT) of the chest revealed multiple bilateral pulmonary nodules and a CT of the abdomen and pelvis showed a large heterogeneous soft tissue mass measuring 9.3×8×10.5 cm. A CT-guided core needle biopsy of a lung nodule was performed and the findings were consistent with the diagnosis of metastatic choriocarcinoma. Magnetic resonance imaging (MRI) of the brain was negative for metastatic disease. Tumor markers were significant for a markedly elevated beta human chorionic gonadotropin (B-hCG) of 104 712 mIU/mL. He was diagnosed with a stage IIIC germ cell tumor, further classified as a primary retroperitoneal choriocarcinoma with lung metastasis, and was started on urgent inpatient chemotherapy. CONCLUSIONS Due to the poor outcomes associated with extragonadal choriocarcinoma, it is important to promptly and correctly identify this malignancy in order to initiate treatment in a timely manner. The following case report explores the histopathologic characterization of this malignancy and describes the clinical course and outcomes from treatment for this patient.

Methaemoglobinaemia from Vagisil creme in a 50-year-old woman.

Methaemoglobinaemia is a life-threatening condition that results from increased methaemoglobin production. As methaemoglobin is unable to reversibly bind to oxygen potentially lethal hypoxia and functional anaemia can occur. Benzocaine can be used as a topical anaesthetic and can be found in many nonprescription preparations marketed for self-application. It is known to cause methaemoglobinaemia in rare cases but most reports describe the complication occurring during endoscopy procedures. Methaemoglobinaemia occurring after topical benzocaine use on the perineum of a perimenopausal woman is exceedingly rare. A 50-year-old woman with methaemoglobinaemia secondary to the perineal application of over-the counter Vagisil (benzocaine 20% and resorcinol 3%- an antiseptic and disinfectant, respectively) presented to the emergency department. She had been using Vagisil for severe, chronic vaginal itching. While methaemoglobinaemia secondary to excessive use of over-the-counter medications such as Vagisil creme is exceedingly rare, it should be included in the differential diagnosis.

Extensive catastrophic thromboses from elevation of factor VIII.

Catastrophic thrombotic syndrome, otherwise known as thrombotic storm (TS) is an extreme prothrombotic clinical syndrome that presents as rapid onset of multiple thromboembolic events affecting a large variety of vasculature. In recent studies, there has been a correlation of high plasma levels of factor VIII with thrombotic events. We present the case of a young man who exhibited multi-organ failure due to thrombotic storm. A 38-year-old male presented to the emergency department for progressive dyspnea and was diagnosed to have pulmonary embolism. The patient developed respiratory distress requiring intubation and was diagnosed with both an ST-elevation myocardial infarction and right cerebral infarction during the hospital course. The patient expired and autopsy revealed the cause of death to be myocardial, cerebral and renal infarction from widespread vascular thrombosis. Autopsy revealed cause of death to be elevated factor VIII associated thrombotic coagulopathy. Factor VIII level upon autopsy was 375% (55-200%). Although TS is rare, it can be lifethreatening if not recognized early. Survival depends on the prompt initiation and duration of anticoagulation.

Hypercalcemia Resulting from Necrotizing Leiomyoma in a Pregnant Female.

BACKGROUND Hypercalcemic crisis is defined as a serum calcium level >14 mg/dL in a symptomatic patient. While severe hypercalcemia during pregnancy is rare, it poses a life-threatening risk to both mother and fetus. Hypercalcemia in association with a benign tumor such as a leiomyoma is exceedingly rare. CASE REPORT A 38-year-old primagravida at 31.2 week's gestation conceived by in vitro fertilization presented to the emergency department for complaints of nausea, vomiting, and epigastric abdominal pain. Her fetal monitor strip was reassuring. A complete metabolic panel on admission was significant for severely elevated calcium of 15.9 mg/dL (8.6-10.3 mg/dL) and an elevated lipase of 1457U/L (11-82 U/L). She was started on aggressive intravenous fluid resuscitation, but became confused and lethargic, unarousable to verbal stimuli, as a result of hypercalcemia. Computed tomography (CT) scan of the abdomen and pelvis revealed a heterogeneously enhancing, placental-appearing soft tissue mass extending posteriorly and to the right that measured 2414 cm. The patient subsequently underwent planned low transverse cesarean delivery and exploratory laparotomy for myomectomy with removal of a 2834-g benign leiomyoma measuring 19.018.514.0 cm. Her serum parathyroid hormone-related protein (PTHrP) was elevated to 9.6 pmol/L (<4.2 pmol/L). The patient's calcium normalized to 9.8 mg/dL (8.6-10.3mg/dL) immediately following surgery. CONCLUSIONS Leiomyoma as a cause of hypercalcemia should be included in the differential diagnosis because surgical removal of leiomyoma is curative. Particularly in pregnant patients, for whom medical therapies for hypercalcemia are limited and those available can result in complications, early identification and surgical resection can be life saving.

T Cell Primary Effusion Lymphoma in an HIV-Negative Man with Liver Cirrhosis.

BACKGROUND Primary effusion lymphoma (PEL) is a rare and aggressive non-Hodgkin lymphoma (NHL) that is responsible for 1% of all lymphomas not related to human immunodeficiency virus (HIV). PEL is characterized by human herpesvirus-8 (HHV-8) positivity in the absence of overt tumor burden that does not exhibit typical B cell or T cell immunophenotype characteristics. The exact mechanism of development is unknown, but it is hypothesized to develop from post-germinal B cell origin. Although it is most common in HIV patients, other immunocompromising comorbidities can be seen in conjunction with PEL, including liver cirrhosis. CASE REPORT We present the case of a 73-year-old HIV-seronegative man with alcohol-induced liver cirrhosis who was found to have T cell PEL of the pleural space diagnosed by thoracentesis. CONCLUSIONS Little is known regarding oncogenesis of T cell PEL, and few studies exist regarding appropriate treatment regimens for PEL as a whole, prompting need for further investigation and discussion to improve survival rates. Even in the absence of active HIV infection, PEL should be considered as a potential cause of pleural effusion in cirrhotic patients in order to prompt earlier treatment for the best chance of survival.

A Case of Thymic Carcinoma with Bone and Cerebral Metastases Treated with Stereotactic Radiosurgery and Chemotherapy.

BACKGROUND Thymic carcinoma is a rare malignant neoplasm. High-grade thymic carcinoma has a high recurrence rate following surgery, and a low 5-year survival rate. Approximately 30% of patients with thymic carcinoma will be asymptomatic at the time of diagnosis. Extrathoracic metastasis on presentation is uncommon. Treatment of the primary tumor includes surgery, chemotherapy, and fractionated radiation. A rare case of thymic carcinoma that presented with bone and cerebral metastases is reported in a patient who responded well to stereotactic radiosurgery and chemotherapy. CASE REPORT A 63-year-old woman presented to the hospital for evaluation of hip pain. She was diagnosed with a lytic bone lesion of the right femur and brain metastasis. Biopsies from the mediastinal mass and right femur showed histological features consistent with carcinoma. Immunohistochemistry showed positive immunostaining of the tumor cells for the c-kit receptor (CD117) and CD5, supporting a diagnosis of stage IVb thymic carcinoma. Treatment included stereotactic radiosurgery, which delivered multiple radiation beams to the tumor tissue from different directions to target the tumor without affecting normal tissues. She was treated as an outpatient with carboplatin and taxol after stereotactic radiosurgery. The patient recovered well following treatment. CONCLUSIONS A case of thymic carcinoma with bone and cerebral metastases was successfully treated with stereotactic radiosurgery and chemotherapy.

Plummer-Vinson Syndrome and Heart Failure: An Unusual Association in an African American Woman.

BACKGROUND Plummer-Vinson syndrome (PVS) is a rare disorder composed of the triad of dysphagia, iron-deficiency anemia (IDA), and esophageal webs. It is most prevalent in middle-aged white women, and the dysphagia often improves when the anemia is treated. It is well established that chronic hypertension can lead to congestive heart failure (CHF). While IDA is frequently found concomitantly with CHF, there have been no reported cases of new-onset CHF with anemia presenting as PVS. CASE REPORT We present the case of a 48-year-old African American woman with symptomatic anemia and new-onset congestive heart failure secondary to hypertension, who presented with the classic symptoms of PVS. CONCLUSIONS CHF with accompanying IDA may be an independent risk factor for the development of PVS. At the very least, there is an association between CHF-induced IDA and PVS. Patients presenting with CHF with symptoms of dysphagia should be considered at risk for the syndrome, and endoscopy may be warranted. Treatment for PVS includes iron replacement, and in some cases requires mechanical dilation.

Successful Use of Adjunctive Red Blood Cell Exchange Therapy for Treatment of an Acute Hemolytic Reaction After ABO-Incompatible Red Blood Cell Transfusion.

An acute hemolytic transfusion reaction is a potentially fatal complication resulting from the transfusion of mismatched blood products. Symptoms vary from mild to severe depending on how much incompatible antigen was transfused and the nature of the recipient's antibodies. There is no consensus agreement of appropriate management other than discontinuing the transfusion and basic supportive methods including adjunctive pharmacologic agents. A 40-year-old male presented with a gunshot wound to the upper torso. During surgery, the O+ patient lost 1.3 L of blood and postoperatively was inadvertently given one unit of A+ packed red blood cells. The blood bank noticed the error and notified the floor within the hour. An acute hemolytic transfusion reaction had progressed to shock and disseminated intravascular coagulation within hours. The clinical course continued to decline despite a norepinephrine drip and a red blood cell exchange transfusion was implemented within 5 h of the mismatched transfusion. The patient's hematological parameters and clinical markers improved and he was eventually discharged in stable condition. An adjunctive red blood cell exchange transfusion may be useful when treating an ABO-incompatible acute hemolytic transfusion reaction if there has been a large volume mismatched transfusion and a poor clinical response to basic supportive methods.

Black Esophagus: A Rare Case of Acute Esophageal Necrosis Induced by Diabetic Ketoacidosis in a Young Adult Female.

BACKGROUND: Acute esophageal necrosis is an uncommon clinical disorder diagnosed on endoscopy as a black esophagus. It has a multifactorial etiology that probably represents a combination of poor nutritional status, gastric outlet obstruction, and ischemia secondary to hypoperfusion of the distal esophagus. It typically occurs in older males with comorbidities. CASE: A 37-year-old woman presented with diabetic ketoacidosis and hematemesis. At esophagogastroduodenoscopy acute esophageal necrosis was diagnosed. The treatment included fluid and electrolyte management, insulin, and a proton pump inhibitor. She improved and left the hospital on day 3. CONCLUSION: Diabetic ketoacidosis can result in a profound osmotic diuresis, fluid loss, and hypoperfusion of the distal esophagus. This condition can then lead to ischemic injury and acute esophageal necrosis. Awareness of the possibility of its presence in young women with hematemesis and poorly controlled diabetes is important since early identification with esophagogastroduodenoscopy is necessary to prevent serious postnecrotic complications.