In Behcet's disease, thrombocytopenia has rarely been reported in association with the hemolytic uremic syndrome, thrombotic thrombocytopenic purpura or in association with cyclosporine or chlorambucil in the treatment of ocular inflammatory disease and meningoencephalitis. In this paper we report a case of thrombocytopenia in a 33-year-old female with Behcet's disease who has taken no medications for three years. After history taking, physical examination, routine laboratory and bone marrow examination, we diagnosed her case as idiopathic thrombocytopenic purpura (ITP). She recovered with high dose steroid treatment. To our knowledge, this is the first report having ITP in a patient with Behcet's disease.
Adult ; Bone Marrow Examination ; Chlorambucil ; Cyclosporine ; Female ; Hemolytic-Uremic Syndrome ; Humans ; Meningoencephalitis ; Physical Examination ; Purpura, Thrombocytopenic, Idiopathic* ; Purpura, Thrombotic Thrombocytopenic ; Thrombocytopenia

We describe here the case a patient with advanced cervix carcinoma and who developed idiopathic thrombocytopenic purpura (ITP). A 63-year-old woman with stage IV squamous cell carcinoma of the uterine cervix and that was complicated by hydronephrosis was treated palliatively with 45Gy of external beam radiation to the pelvis. About 3 years later, she developed hematochezia and severe thrombocytopenia. The laboratory examinations showed no evidence of thrombotic thrombocytopenic purpura or disseminated intravascular coagulopathy, and she was positive for serum anti-platelet antibodies. On the bone marrow examination, there was a normal number and morphology of megakaryocytes with no evidence of malignant cell infiltration. We made the clinical diagnosis of ITP, and the intravenous immunoglobulin and steroid therapy was successful. This case suggests the possibility that ITP can occur in association with advanced cervix carcinoma.
Antibodies ; Bone Marrow Examination ; Carcinoma, Squamous Cell ; Cervix Uteri ; Female ; Gastrointestinal Hemorrhage ; Humans ; Hydronephrosis ; Immunoglobulins ; Megakaryocytes ; Middle Aged ; Pelvis ; Purpura, Thrombocytopenic, Idiopathic ; Purpura, Thrombotic Thrombocytopenic ; Thrombocytopenia

Thromobotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS), characterized by microangiopathic hemolytic anemia, thrombocytopenia, fever, central nervous system abnormalities, and renal dysfunction, is severe multisystem disorder. TTP-HUS occurs predominantly in the reproductive aged-women, associated with poor prognosis. Although the morbidity and mortality have been significantly decreased by using plasma exchange therapy, refractory TTP-HUS remains a tremendous problem. It is crucial to differentiate other microangiopathic hemolytic anemia disease with a confusing presentation and to perform the immediate plasmapheresis. We have experienced three cases, which were initially diagnosed as HELLP syndrome or immune thrombocytopenic purpura. Despite of aggressive plasmapheresis, two women died. We present these cases with a review of the literature on pregnancy-associated thrombotic microangiopathy, including ADAMTS-13 activity assay as a new diagnostic test.
Anemia, Hemolytic ; Central Nervous System ; Diagnostic Tests, Routine ; Female ; Fever ; HELLP Syndrome ; Humans ; Plasma Exchange ; Plasmapheresis ; Pre-Eclampsia ; Pregnancy ; Pregnancy Complications ; Prognosis ; Purpura, Thrombocytopenic, Idiopathic ; Purpura, Thrombotic Thrombocytopenic ; Thrombocytopenia ; Thrombotic Microangiopathies

Objective: To report the clinical manifestations and laboratory features of five patients with congenital thrombotic thrombocytopenic purpura (cTTP) and explore its standardized clinical diagnosis and treatment along with a review of literature. Methods: Clinical data of patients, such as age of onset, disease manifestation, personal history, family history, and misdiagnosed disease, were collected. Treatment outcomes, therapeutic effects of plasma infusion, and organ function evaluation were observed. The relationship among the clinical manifestations, treatment outcomes, and ADAMTS13 gene mutation of patients with cTTP was analyzed. Additionally, detection of ADAMTS13 activity and analysis of ADAMTS13 gene mutation were explored. Results: The age of onset of cTTP was either in childhood or adulthood except in one case, which was at the age of 1. The primary manifestations were obvious thrombocytopenia, anemia, and different degrees of nervous system involvement. Most of the patients were initially suspected of having immune thrombocytopenia. Acute cTTP was induced by pregnancy and infection in two and one case, respectively. ADAMTS13 gene mutation was detected in all cases, and there was an inherent relationship between the mutation site, clinical manifestations, and degree of organ injury. Therapeutic or prophylactic plasma transfusion was effective for treating cTTP. Conclusions: The clinical manifestations of cTTP vary among individuals, resulting in frequent misdiagnosis that delays treatment. ADAMTS13 activity detection in plasma and ADAMTS13 gene mutation analysis are important bases to diagnose cTTP. Prophylactic plasma transfusion is vital to prevent the onset of the disease.
Female ; Pregnancy ; Humans ; Adult ; Blood Component Transfusion ; Plasma ; Purpura, Thrombotic Thrombocytopenic/therapy* ; Mutation ; Purpura, Thrombocytopenic, Idiopathic ; ADAMTS13 Protein/therapeutic use*

No abstract available.
Purpura ; Purpura, Thrombocytopenic, Idiopathic

Child ; Humans ; Purpura, Thrombocytopenic, Idiopathic ; Thrombocytopenia

No abstract available.
Pregnancy* ; Purpura, Thrombotic Thrombocytopenic*

No abstract available.
Purpura, Thrombotic Thrombocytopenic*

Thrombotic thrombocytopenic purpura (TTP) and the related hemolytic uremic syndrome (HUS) are disorders characterized by thrombocytopenia, microangiopathic hemolytic anemia, a variable degree of impairment of renal function and fluctuating neurological symptoms, which are thought to be due to platelet activation and subsequent formation of thrombi in the microcirculation. The fact that there was no clear-cut clinical and laboratory features that differentiate HUS from TTP has lead to view these two syndromes as a clinical continuum. Microvascular thrombosis is the typical lesion and closely related with endothelial injury and platelet activation. Pathologic alterations of the brain parenchyma are mainly manifested by small multiple infarcts. Numerous cases of CNS complications of these syndromes have been evaluated by using CT, but few reports have mentioned the MR findings. We experienced a case of TTP-HUS that had clinical features of cortical blindness and the brain lesion was confirmed by MRI showing cerebral infarct at the occipital area but it was reversible course. So we report this case with a brief review of literature.
Anemia, Hemolytic ; Blindness, Cortical* ; Brain ; Hemolytic-Uremic Syndrome* ; Magnetic Resonance Imaging* ; Microcirculation ; Platelet Activation ; Purpura, Thrombotic Thrombocytopenic* ; Thrombocytopenia ; Thrombosis

The postpartum thrombotic thrombocytopenic purpura-hemolytic uremic syndrome is a rare complication of normal pregnancy and delivery that is described as a constellation of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. We report a case in patient with postpartum thrombotic thrombocytopenic purpura-hemolytic uremic syndrome who was successfully treated with plasama exchange and prednisolone. Relevant literature was reviewed.
Acute Kidney Injury ; Anemia, Hemolytic ; Hemolytic-Uremic Syndrome ; Humans ; Postpartum Period* ; Prednisolone ; Pregnancy ; Purpura, Thrombotic Thrombocytopenic ; Thrombocytopenia