Adolescent ; Adult ; Female ; Humans ; Lupus Erythematosus, Systemic ; complications ; Male ; Purpura, Thrombotic Thrombocytopenic ; diagnosis ; etiology ; therapy

Thrombotic thrombocytopenic purpura (TTP) is a rapidly progressive hematological syndrome defined by the pentad of thrombocytopenia, microangiopathic hemolytic anemia, neurologic abnormalities, fever and renal dysfunction. TTP has been associated with major surgical procedures and specific medications. However, there is no known previously reported case in which acute TTP occurred after a percutaneous coronary intervention (PCI). We report a case of TTP after a PCI, that presented with the pentad of symptoms, as well as hepatitis and pancreatitis.
Stents/*adverse effects ; Purpura, Thrombotic Thrombocytopenic/diagnosis/*etiology ; Humans ; Female ; Angioplasty, Transluminal, Percutaneous Coronary/*adverse effects ; Aged

Behcet's disease with concomitant thrombotic thrombocytopenic purpura (TTP), coronary artery stenosis and coronary artery pseudo aneurysm is rare. Here we report a case of Behcet's disease with several cardiovascular complications, namely: pericarditis, deep vein thrombosis (DVT), TTP, coronary artery stenosis, and a coronary artery pseudo aneurysm. A 37-year-old female presented with sudden dyspnea and syncope at our emergency room and underwent pericardiectomy and pericardial window formation for the diagnosis of cardiac tamponade with acute hemorrhagic pericarditis. Thereafter, TTP and DVT complicated her illness. After confirmation of Behcet's disease on the basis of a history of recurrent oral and genital ulcers and erythema nodosum, remission was achieved after treatment with methylprednisolone pulse therapy, colchicine, catheter directed thrombolysis and thrombectomy. However, whilst maintaining anticoagulation therapy, a newly developed pericardial aneurysmal dilatation was noted on follow-up radiologic evaluation. Further evaluation revealed right coronary artery stenosis and a left coronary artery pseudo aneurysm; these additional problems were treated with the nonsurgical insertion of an endovascular graft stent . At the time of writing three months later after stent insertion, the aneurysm has continued to regress and no additional complications have intervened with combined immunosuppressive therapy.
Venous Thrombosis/diagnosis/*etiology ; Purpura, Thrombotic Thrombocytopenic/diagnosis/*etiology ; Pericarditis/diagnosis/*etiology ; Humans ; Female ; Echocardiography ; Coronary Vessels/*physiopathology ; Coronary Stenosis/diagnosis/*etiology ; Cardiac Tamponade/diagnosis/*etiology ; Behcet Syndrome/*complications/diagnosis ; Aneurysm, False/diagnosis/*etiology ; Adult

Thrombotic microangiopathy (TMA) is a lethal transplantation-associated complication which exactly likes acute intestinal graft-versus-host disease (GVHD) in the clinical manifestation. 373 consecutive patients with hematological diseases received family HLA matched or mismatched HCT from May, 2002 to July, 2004. To analyse the clinical and pathological characteristics of TMA, 30 patients who suffered from severe diarrhea and received colonoscopic examination and gut biopsy were retrospectively analyzed. The results indicated that 7 patients originally diagnosed as gut GVHD showed the pathological evidence of enteric TMA. The incidence of TMA was 7 out of 30 specimen (23.3%). Pathological evidence of enteric TMA shown microvascular disorder characterized by thrombus in the capillary without infiltration of lymphocytes and perivascular hemorrhages in the mucosa, swelling and focal denudation of epithelial cells. All patients with TMA were associated with cytomegalovirus (CMV) antigenemia/disease. Among these patients, 4 cases, who only showed TMA without the evidence of gut GVHD pathologically, displayed treatment-resistant bloody diarrhea, renal failure, veno-occlusive disease, hemorrhagic cystitis, hemolytic anemia as well as thrombocytopenia. But the other 3 cases, with co-existence of both TMA and GVHD pathological characteristics had better treatment response. Survival analysis indicated that 3 patients with TMA-GVHD survived for 461 to 536 days but three out of four TMA patients died from VOD with liver failure as well as multiple organ failure during 101 to 254 days after HCT. In conclusion, to better diagnose those patients with severe and refractory diarrhea following HCT, pathological examination may indicate crux evidence to identify intestinal TMA from gut GVHD. Furthermore, this primary report has first evidenced that TMA and TMA-GVHD are two pathologically well-recognized subtypes with the difference between the pathological characteristics, treatment response and clinical outcomes.
Graft vs Host Disease ; etiology ; Hematopoietic Stem Cell Transplantation ; adverse effects ; Humans ; Intestinal Diseases ; diagnosis ; etiology ; pathology ; Purpura, Thrombotic Thrombocytopenic ; diagnosis ; etiology ; pathology ; Reference Standards ; Retrospective Studies ; Thrombosis ; diagnosis ; etiology ; pathology

Acute Kidney Injury ; etiology ; Adult ; Diagnosis, Differential ; Female ; HELLP Syndrome ; pathology ; Hemolytic-Uremic Syndrome ; complications ; pathology ; therapy ; Humans ; Plasma Exchange ; Postpartum Period ; Pregnancy ; Purpura, Thrombotic Thrombocytopenic ; pathology ; Young Adult

Microangiopathic hemolytic anemia (MAHA) occurs occasionally as a paraneoplastic syndrome in some solid tumors, but MAHA accompanied by signet ring cell carcinoma of an unknown origin is very rare. In this study, we present the case of an 80-yr-old man who was admitted to the hospital because of a 1-month history of lower back pain and dyspnea. He was diagnosed with MAHA on the basis of the laboratory findings that revealed anemia with schistocytes, decreased haptoglobin levels, and a negative direct Coombs' test. Bone marrow examination, which was performed because of the progression of anemia, revealed bone marrow metastases of signet ring cell carcinoma with extensive bone marrow necrosis. However, the primary origin of this signet ring cell carcinoma was not found. When the cause of progressive MAHA is unknown, the possibility of cancer-associated MAHA must be excluded by performing additional tumor workup, including the detection of tumor markers, gastric and colorectal endoscopic examinations, bone marrow examinations, and positron emission tomography-computed tomography or bone scans.
Aged, 80 and over ; Bone Marrow Neoplasms/complications/*diagnosis/pathology ; Carcinoma, Signet Ring Cell/complications/*diagnosis/pathology ; Endoscopy, Gastrointestinal ; Haptoglobins/metabolism ; Humans ; Immunohistochemistry ; Male ; Necrosis/etiology ; Neoplasm Metastasis ; Positron-Emission Tomography ; Purpura, Thrombotic Thrombocytopenic/*diagnosis/etiology ; Tomography, X-Ray Computed ; Tumor Markers, Biological/analysis

Microangiopathic hemolytic anemia (MAHA) occurs occasionally as a paraneoplastic syndrome in some solid tumors, but MAHA accompanied by signet ring cell carcinoma of an unknown origin is very rare. In this study, we present the case of an 80-yr-old man who was admitted to the hospital because of a 1-month history of lower back pain and dyspnea. He was diagnosed with MAHA on the basis of the laboratory findings that revealed anemia with schistocytes, decreased haptoglobin levels, and a negative direct Coombs' test. Bone marrow examination, which was performed because of the progression of anemia, revealed bone marrow metastases of signet ring cell carcinoma with extensive bone marrow necrosis. However, the primary origin of this signet ring cell carcinoma was not found. When the cause of progressive MAHA is unknown, the possibility of cancer-associated MAHA must be excluded by performing additional tumor workup, including the detection of tumor markers, gastric and colorectal endoscopic examinations, bone marrow examinations, and positron emission tomography-computed tomography or bone scans.
Aged, 80 and over ; Bone Marrow Neoplasms/complications/*diagnosis/pathology ; Carcinoma, Signet Ring Cell/complications/*diagnosis/pathology ; Endoscopy, Gastrointestinal ; Haptoglobins/metabolism ; Humans ; Immunohistochemistry ; Male ; Necrosis/etiology ; Neoplasm Metastasis ; Positron-Emission Tomography ; Purpura, Thrombotic Thrombocytopenic/*diagnosis/etiology ; Tomography, X-Ray Computed ; Tumor Markers, Biological/analysis