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

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

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

Thrombotic thrombocytopenic purpura(TTP) is a clinical syndrome of unknown etiology and characterized by microangiopathic hemolytic anemia, thrombocytopenia, fluctuating neurological status, renal dysfunction and fever. Systemic lupus erythromatosus(SLE) is also multisystemic disease that some of clinical features may mimic TTP. Therefore both diseases have led to diagnostic confusion. We experienced two cases with SLE who subsequently or initially developed TTP. In case 1, a 44-year old woman had 1-year previous history of SLE and presented with dyspnea. After diagnosis of thrombotic microangiopathy by renal biopsy, she was managed with steroid, cyclophosphamide pulse therapy, fresh frozen plasma infusion and plasmapheresis. She was treated by aggressive treatment; nevertheless, she died on 15th admission day. In case 2, a 22-year old man was admitted because of nausea and vomiting. SLE with TTP was diagnosed by ARA criteria and the finding of microangiopathic hemolytic anemia. He was treated with plasmapheresis, fresh frozen plasma infusion and steroid therapy. He showed clinical response to the therapy, and has shown no recurrence of disease until now on. In conclusion, we suggest that early diagnosis and prompt therapy such as plasmapheresis and plasma infusion are very important in SLE with TTP.
Adult ; Anemia, Hemolytic ; Biopsy ; Cyclophosphamide ; Diagnosis ; Dyspnea ; Early Diagnosis ; Female ; Fever ; Humans ; Nausea ; Plasma ; Plasmapheresis ; Purpura, Thrombocytopenic* ; Purpura, Thrombotic Thrombocytopenic ; Recurrence ; Thrombocytopenia ; Thrombotic Microangiopathies ; Vomiting ; Young Adult

This study was aimed to investigate the detection and diagnosis of the neonatal alloimmune thrombocytopenia purpura (NAITP) caused by anti-HPA-5b antibody. The platelet count and clinical manifestation in the newborn were examined. The HPA-1-21bw genotypes of the newborn and her parents were detected by multiple-PCR and DNA sequencing. The HPA-specific antibody in the sera of newborn and her mother were detected and identified by flow cytometry (FCM) and monoclonal antibody-specific immobilization of platelet antigens (MAIPA). The results indicated that the clinical manifestations of the newborn were lighter. The HPA genotyping showed that the genotype of the newborn was HPA-5ab, while that of her mother and father were HPA-5aa and HPA-5ab, respectively. The antibody against the platelet of newborn's father existed in the newborn's mother sera. The HPA antibody of the mother was identified as anti-HPA-5b. It is concluded that the newborn with neonatal alloimmune thrombocytopenia purpura was caused by the antibody against HPA-5b.
Antigens, Human Platelet ; genetics ; China ; Female ; Genotype ; Humans ; Infant, Newborn ; Purpura, Thrombocytopenic, Idiopathic ; diagnosis ; genetics ; Thrombocytopenia, Neonatal Alloimmune ; diagnosis ; genetics

OBJECTIVE: The incidence of idiopathic thrombocytopenic purpura (ITP) is greatest in female during their childbearing years, so the concurrence of pregnancy and ITP is not unusual. Numerous studies have examined the outcomes of newborns, whereas fewer studies have been conducted with regard to the morbidity of obstetric patients with ITP. This study was aimed to find the outcome of pregnancy combined with ITP and the influence of the pregnancy on the severity of this disease. METHODS: From January 1996 to December 2005, a total of 62 pregnant women with ITP and their 73 deliveries were recruited for the study. Among them, 38 were diagnosed with ITP during pregnancy and the other 24 had pre-existing ITP before pregnancy. RESULTS: The severity of thrombocytopenia was exacerbated during pregnancy, but recovered to a level of non-pregnant period after delivery in most cases. The outcome of pregnancy of all the patients was uneventful except each one case of fetal demise at 35 gestational weeks and preterm delivery at 30 gestational weeks. One patient suffered from multiple subdural hemorrhage during pregnancy, which was spontaneouly recovered. Twenty newborns (27.8%) had transient congenital thrombocytopenia and 18 of them required treatment for hemostatic impairment. CONCLUSION: For women with ITP, Pregnancy can affect the severity of ITP, but life-threatening complication was almost lacking. Although, in not a few cases, there may need to treat both mothers and infants to raise their platelet counts, most mothers with ITP can proceed with their pregnancies and delivery healthy infant without complication.
Female ; Hematoma, Subdural ; Humans ; Incidence ; Infant ; Infant, Newborn ; Mothers ; Platelet Count ; Pregnancy* ; Pregnant Women ; Purpura* ; Purpura, Thrombocytopenic, Idiopathic ; Thrombocytopenia* ; Thrombocytopenia, Neonatal Alloimmune

Diagnosis of thrombotic microangiopathy (TMA) is challenging due to its close association with other forms of microangiopathic hemolytic anemia, such as malignant hypertension and disseminated intravascular coagulation, and because other manifestations including cytopenia and acute kidney injury are manifestations of other medical comorbidities. Further challenges for accurate diagnosis include distinguishing between primary and secondary TMA, as well as between hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP). TTP is typically differentiated from HUS by the presence of more severe thrombocytopenia, along with a higher frequency of altered mental status with relatively preserved renal function. However, the clinical course can vary among patients, requiring polymerase chain reaction testing of patient stools for enterohemorrhagic Escherichia coli and a disintegrin and metalloproteinase with thrombospondin type 1 motif 13 (ADAMTS13) assay. To reduce the mortality rate, prompt initiation of plasmapheresis is important in cases where TPP cannot be excluded. Future advances enabling more rapid testing for ADAMTS13 levels will reduce the need for unnecessary plasmapheresis, so that treatment strategy can be more optimized.
Acute Kidney Injury ; Anemia, Hemolytic ; Comorbidity ; Diagnosis ; Diagnosis, Differential ; Disseminated Intravascular Coagulation ; Enterohemorrhagic Escherichia coli ; Hemolytic-Uremic Syndrome ; Humans ; Hypertension, Malignant ; Mortality ; Plasma Exchange ; Plasmapheresis ; Polymerase Chain Reaction ; Purpura, Thrombotic Thrombocytopenic ; Thrombocytopenia ; Thrombospondins ; Thrombotic Microangiopathies

We report the case of a 14-year-old girl, diagnosed with atypical thrombotic microangiopathy (TMA). The patient presented with persistent fever, nausea, and newly developed peripheral edema. Her laboratory findings indicated chronic anemia with no evidence of hemolysis, thrombocytopenia, or elevated serum creatinine level. A few days after hospitalization, acute renal failure and fever worsened, and proteinuria developed. On day 40 of hospitalization, she experienced a generalized tonic seizure for 5 min, accompanied by renal hypertension. Brain magnetic resonance imaging revealed posterior reversible leukoencephalopathy syndrome. After steroid pulse therapy, a renal biopsy was performed because of delayed recovery from thrombocytopenia. The biopsy findings showed features of thrombotic microangiopathic hemolysis with fibrinoid change restricted. Current diagnostic criteria for TMA have focused on thrombotic thrombocytopenic purpura and hemolytic uremic syndrome, and diagnosis is based on the clinical presentation and etiology, with the consequence that idiopathic and atypical forms of TMA can be overlooked. Developing effective tools to diagnose TMA, such as studying levels of ADAMTS13 or testing for abnormalities in the complement system, will be the first step to improving patient outcomes.
Acute Kidney Injury ; Adolescent ; Anemia ; Biopsy ; Brain ; Complement System Proteins ; Creatinine ; Diagnosis ; Edema ; Female ; Fever ; Hemolysis ; Hemolytic-Uremic Syndrome ; Hospitalization ; Humans ; Hypertension, Renal ; Leukoencephalopathies ; Magnetic Resonance Imaging ; Nausea ; Proteinuria ; Purpura, Thrombotic Thrombocytopenic ; Resin Cements ; Seizures ; Thrombocytopenia ; Thrombotic Microangiopathies*

We report the case of a 14-year-old girl, diagnosed with atypical thrombotic microangiopathy (TMA). The patient presented with persistent fever, nausea, and newly developed peripheral edema. Her laboratory findings indicated chronic anemia with no evidence of hemolysis, thrombocytopenia, or elevated serum creatinine level. A few days after hospitalization, acute renal failure and fever worsened, and proteinuria developed. On day 40 of hospitalization, she experienced a generalized tonic seizure for 5 min, accompanied by renal hypertension. Brain magnetic resonance imaging revealed posterior reversible leukoencephalopathy syndrome. After steroid pulse therapy, a renal biopsy was performed because of delayed recovery from thrombocytopenia. The biopsy findings showed features of thrombotic microangiopathic hemolysis with fibrinoid change restricted. Current diagnostic criteria for TMA have focused on thrombotic thrombocytopenic purpura and hemolytic uremic syndrome, and diagnosis is based on the clinical presentation and etiology, with the consequence that idiopathic and atypical forms of TMA can be overlooked. Developing effective tools to diagnose TMA, such as studying levels of ADAMTS13 or testing for abnormalities in the complement system, will be the first step to improving patient outcomes.
Acute Kidney Injury ; Adolescent ; Anemia ; Biopsy ; Brain ; Complement System Proteins ; Creatinine ; Diagnosis ; Edema ; Female ; Fever ; Hemolysis ; Hemolytic-Uremic Syndrome ; Hospitalization ; Humans ; Hypertension, Renal ; Leukoencephalopathies ; Magnetic Resonance Imaging ; Nausea ; Proteinuria ; Purpura, Thrombotic Thrombocytopenic ; Resin Cements ; Seizures ; Thrombocytopenia ; Thrombotic Microangiopathies*

Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy characterized by endothelial cell damage, resulting in microangiopathic hemolytic anemia, thrombocytopenia, and various degrees of neurological and renal impairment caused by microvascular thrombi. It is rare in children and frequently follows a fatal course. TTP is divided into 2 types: one is inherited and associated with ADAMTS-13 gene mutations and the other is acquired and associated with anti-ADAMTS-13 autoantibodies. The measurement of ADAMTS-13 activity in plasma, identification of ADAMTS-13 circulating inhibitor, anti-ADAMTS-13 IgG, and ADAMTS-13 gene sequencing are crucial to the diagnosis of TTP. Plasma exchanges are the first-line treatment for acquired TTP, combined with steroids and immunosuppressive drugs. Here, we describe the case of an adolescent patient with TTP, confirmed by decreased level of ADAMTS-13 activity and an increased level of ADAMTS-13 inhibitor, who was successfully treated by plasma exchanges.
Adolescent ; Anemia, Hemolytic ; Autoantibodies ; Child ; Endothelial Cells ; Humans ; Immunoglobulin G ; Plasma ; Plasma Exchange ; Purpura ; Purpura, Thrombotic Thrombocytopenic ; Steroids ; Thrombocytopenia ; Thrombotic Microangiopathies ; Thymine Nucleotides