Introduction: Plasmacytoid dendritic cell leukemia is a rare subtype of acute leukemia, which has recently been established as a distinct pathologic entity that typically follows a highly aggressive clinical course in adults. The aim of this report is to present a case of plasmacytoid dendritic cell leukemia due to its rarity and difficulty to recognize and diagnose it.
Case report: We present a case of a 67 year-old man who presented multiple subcutaneous lesions on his face, neck, chest and upper extremities with reddish-brown, brown colour. In the bone marrow aspirate 83% of the blast cells were found. Immunophenotypically the blasts were positive for CD4, CD56, CD123 (high intensity), CD36, CD22, CD10 (10.42%), CD33, HLA-DR, CD7 (9.24%), CD38 (34.8%) and negative for CD13, CD64, CD14, CD16, CD15, CD11b, CD11c, CD3, CD5, CD2, CD8, CD19, CD20, CD34. The skin biopsy showed lymphohistiocytoid infiltration in the dermis. The patient was diagnosed with acute plasmacytoid dendritic cell leukemia and received polychemotherapy with rapid response of skin lesions and blastic infiltration of the bone marrow. After 3 courses of polychemotherapy the cutaneous lesions reappeared and multiplied. The blast infiltration in the bone marrow increased to 70%. A more aggressive polychemotherapy regimen was administered, but the patient presented serious complications (febrile neutropenia) and died in septic shock 8 months after the initiation of treatment.
Conclusions: Immunophenotyping of blasts cells is indispensable in the diagnosis of plasmacytoid dendritic cell leukemia. The CD4+, CD56+, lin-, CD123 ++high, CD11c-, CD36+, HLA-DR+, CD34-, CD45+ low profile is highly suggestive for pDCL. The outcome of plasmacytoid dendritic cell leukemia is poor. Despite the high rate of initial response to treatment, early relapses occur and the patients die of disease progression.
Category Archives: Case Report
Tattoo-Associated Skin Reactions — Clinical Cases
Tatooing has been practiced for thousands of years. It has become a common practice for people of Western countries in the last 10–20 years, where approximately 3–5% of the population has at least one tattoo. Various pigmented substances introduced into the skin may cause the occurrence of adverse irritative, immunological, infectious or other reactions of the skin. We present three clinical cases with adverse reactions after tattooing.
Spigelian Port-site Hernia, a Complication after Laparoscopic Cholecystectomy — a Clinical Case Report
Introduction: Spiegelian hernias are rare entities in abdominal wall pathology (2%). They occur in the semilunar line described by Adriaan van den Spiegel. Klinklosch (1764) defined it as a congenital or acquired defect of the transverse abdominal aponeurosis junction with the Douglas arch. Port-site hernias due to wrong placement of laparoscopic trocars in the right abdominal flank are rare, but possible complications of laparoscopic cholecystectomy.
Case presentation: Our observation shows diagnostic and therapeutic aspects in a patient with port-site Spigelian hernia post laparoscopic cholecystectomy admitted in Surgical Clinic 1, County Emergency Clinical Hospital Tîrgu Mureș in the 28.01.2013 – 30.01.2013 period. Following surgery performed using an open approach, postoperative evolution was favorable, with no signs of recurrence at 9 months postoperatively.
Conclusions: Spigelian port-site hernia post laparoscopic cholecystectomy is a very rare entity, iatrogeny being a certainty in its development.
Cor Triatriatum Dexter, a Very Rare Congenital Anomaly Presented in a Complex Pathological Context – Case Presentation
Background: Cor triatriatum dexter is an extremely rare congenital anomaly, and in most cases, without hemodynamic significance. Congenital cytomegalovirus infection is the most common viral infection, the majority of infected infants being asymptomatic and only 5-15% being symptomatic in the neonatal period.
Case report: We present the case of a female infant, aged 3 months. Clinical examination reveals cranial and facial anomalies. The karyotype was normal. Laboratory tests revealed the presence of anti-CMV antibodies in urine. Imaging studies showed cerebral and renal anomalies. Echocardiographic exam revealed a congenital anatomical feature without hemodynamic significance – cor triatriatum dexter.
Conclusions: although cytomegalovirus infection is mostly asymptomatic, it can present as a complex pathological association, in which a congenital cardiac anatomical feature without hemodynamic significance, considered by some authors as an anatomical variant, can appear.
Hilar Cholangiocarcinoma Diagnosed and Treated Early, in Prejaundice Phase
Hilar cholangiocarcinoma, Klatskin tumor or proximal bile duct cancer, is a tumor growing in the right hepatic duct, left hepatic duct or at their confluence. It is a relatively rare but devastating disease. The tight stricture of the biliary ducts and the development of obstructive jaundice are the main characteristics of the disease. In the early phase, symptoms are nonspecific and jaundice is not present, leading to delayed diagnosis and denying the possibility of curative treatment. We present the case of a 74 years old woman who was referred to us with ambiguous symptomatology and without jaundice. The ultrasound and CT scan showed dilation of the left biliary tree, without increase of the cholestatic enzymes. Magnetic resonance cholangiography depicted a tumor in the left hepatic duct (3X3 cm.) with enlargement of the bile ducts above. The surgical treatment consisted of left hepatectomy and hilar lymph nodes dissection. The pathology findings showed a cholangiocarcinoma with a few hilar nodes involvement. Our approach was potentially curative. Unfortunately these situations are seldom because in the majority of cases the patients have obstructive jaundice at presentation and the tumors are unresectable. We consider that a magnetic resonance cholangiography made when we suspect a bile duct tumor, leads us to an early diagnosis and gives us the possibility of a potential curative surgical treatment.
Rare Case of Immature Gastric Teratoma
Introduction: Teratomas are rare and complex tumors with components from more than one of the three germ cell layers. Teratomas range from benign, well-differentiated (mature) cystic lesions to those that are solid and malignant (immature). The incidence of all teratomas is estimated at 1:10,000–1:20,000 newborns. Gastric teratomas represent only 1-2% of all teratomas.
Case presentation: We report a case of gastric teratoma of a 2 month-old boy who presented with abdominal distension. Diagnosis was established by physical examination, ultrasonography and computed tomography. The tumor measured 13/10/5.5 cm and weighted 390 grams and was surgically excised. Histological examination revealed an immature gastric teratoma. We also reviewed existing clinical and genetic data on gastric teratomas. Association of gastric teratomas with other congenital anomalies or tumors is very rare. Reported cases include: Beckwith-Wiedemann syndrome (involved:11p15, IGF2), Hodgkin disease (developed 3 yrs. post-resection) and focal neuroblastoma. Recent theories include extraembryonic cells; also have been hypothesized to originate from pluripotent cells present in the gastric wall.
Conclusion: Gastric teratomas are extremely rare tumors. Complete resection induce a good outcome.
Cephalic Duodeno-Pancreatectomy With Pancreatic-Gastric Anastomosis With Double Purse String, in Patient with Lithiasis and Tumoral Jaundice – Case Report
Introduction: One of the most feared complications after cephalic duodeno-pancreatectomy remains pancreatic fistula. In recent years, various methods of pancreatico-digestive reconstruction were performed in order to reduce the rate of pancreatic fistula. One of these methods is pancreatico-gastric reconstruction by using two purse string threads.
Case report: We present in this article a patient with jaundice with mixed etiology: tumoral and lithiasic. Subjectively, the patient accused sclerose-skin-jaundice, right upper quadrant and epigastric pain, nausea and vomiting. Computed tomography revealed dilatation of intra- and extrahepatic bile ducts, a dilated Wirsung duct and a tumor at the biliopancreatic confluence, leading to a suspicion of vaterian ampulom. Upper endoscopy revealed a tumor protruding in the descending duodenal segment. Intraoperatively a tumor suggestive of vaterian ampulom and duct stones was shown. Surgical treatment consisted of coledocolitotomy, cephalic duodeno-pancreatectomy with pancreatic-gastric anastomosis, performed by using two purse string threads. The postoperative evolution was favorable.
Conclusion: Pancreatico-gastric anastomosis using two purse string threads is a simple, safe and quick procedure, avoiding the application of sutures through the pancreatic parenchyma and thus reducing the rate of pancreatic fistula.
Fanconi Anemia — Case Report of Rare Aplastic Anemia at Child
Introduction: Fanconi anemia is an autosomal recessive disease characterized by congenital abnormalities, defective haematopoiesis, and a high risk of developing acute myeloid leukaemia, myelodysplastic syndrome and cancers. FA was first described in 1927 by the Swiss pediatrician Guido Fanconi. The diagnosis is based on morphological abnormalities, hematologic abnormalities (pancytopenia, macrocytic anemia and progressive bone marrow failure) and genetic tests (cariograma).
Case report: We present the case of a child with Fanconi anemia. Although skin and bone morphological abnormalities were present from birth, diagnosis was suspected at 11 years old.
Conclusions: Fanconi anemia is a heterogeneous condition that can present a variety of congenital defects but invariably results in defective haemopoiesis, which is the major cause of morbidity and mortality.
The Oscar Ramirez Procedure, a Solution for Treating Incisional Hernias with Big Abdominal Wall Defect
Background: Incisional hernias are important complications of abdominal surgery. Normally they are followed by the growth of the hernia sac and an increase of the abdominal wall defect with loss of domain of the herniated organs.
Case report: We report a case of a 51 year old female, admitted in the 1st Surgical Clinic of the County Emergency Clinical Hospital of Tîrgu Mureș with a large median reducible incisional hernia and a wall defect of 10/12 cm. The abdominoplasty was performed using the Oscar Ramirez technique, which consists of a longitudinal incision along the aponeurosis of the external oblique muscle, at approximately 1–2 cm from the external edge of the rectus abdominis. This procedure allows the abdominal wall closure. This case is part of a lot of 4 patients who received this treatment using the aforementioned procedure in the past 2 months. Postoperative recovery was favorable, bowel movements were present on the second day after the surgery, the subcutaneous drains were shortened on the fifth day and removed on the sixth. There were no immediate postoperative complications or up to a month after the surgery. The patient was discharged on the seventh day.
Conclusions: This technique can be used alone in case of large abdominal wall defects, or prequeling an on-lay mesh procedure addressed to a weak abdominal wall, case in which the functional result is superior to a substitutional mesh.
An unusual Case of Cardiac Syncope and Acute Coronary Syndrome – A Case Report
Introduction: We aimed to present a case of acute coronary syndrome with unexpected etiology complicated by syncope and arrhythmias, confirmed by imagistic examinations as cardiac parasitosis. Cardiac parasitic diseases are rare diseases, whose diagnosis and therapy should be adapted to each case. Imaging techniques allow precise diagnosis of cardiac echinococcosis, providing essential structural details on the damage degree of heart structures, allowing optimization of complex treatment in these cases.
Case presentation: A 67-year old, obese and diabetic woman presented with cardiac syncope, arrhythmias and acute chest pain. Imagistic examinations excluded intracoronary thrombosis and confirmed a severe structural damage of myocardial tissue, consisting in replacement of the myocardial structure by many cysts caused by parasitic infestation with echinococcus multilocularis and echinoccocus granulosus originating from the liver. CT scan confirmed severe distruction of the left ventricular myocardium by policysts, that led to thinning of inferior and apical left ventricle wall without any possibility of surgical excision. Therefore a specific chemotherapy with albendazole was initiated. Follow up at 2 months indicated a favorable evolution, with serological decrease of echinococcal antibodies and reduction of cysts volume.
Conclusion: In cases of angina and arrhythmias with non-atherosclerotic etiology, imaging techniques can diagnose the anatomopathological substrate of the disease and represent a valuable tool for the follow up.