Thrombocytopenia in Childhood: An Evaluation of 433 Patients

An evaluation of 433 children with thrombocytopenia is presented. The material comprises all cases diagnosed in Denmark during the period 1959–1969. The incidence was found to be 31.9 cases per 1,000,000 inhabitants 15 years of age per annum with a peak at the age of 3 to 4 years; the majority of the cases was diagnosed in the spring. Preceding infection, usually associated with fever and localized in the upper respiratory tract, was the most common cause. The majority of the congenital cases was hereditary and associated with other malformations involving in particular the immune system. In about half of the cases the course was spontaneous and no treatment was necessary; the remaining patients were treated with glucocorticoids, including 46 patients, who had undergone splenectomy. 22 patients died, including 16 cases due to serious underlying disorders; in 6 cases the cause of death was haemorrhage (mortality rate = 1.4%). At the follow up the platelet count was below 50 mia/1 in 14 patients (4%). No difference was found concerning the platelet count at the follow up between the patients with spontaneous recovery, the patients treated with glucocorticoids and the patients, in whom splenectomy had been performed. During the course the disease autoimmune haemolytic anaemia occurred in 2 patients, whereas no patient developed systemic lupus erythematosus.     

Thrombocytopenia in childhood: an evaluation of 433 patients.

An evaluation of 433 children with thrombocytopenia is presented. The material comprises all cases diagnosed in Denmark during the period 1959-1969. The incidence was found to be 31.9 cases per 1,000,000 inhabitants less than or equal to 15 years of age per annum with a peak at the age of 3 to 4 years; the majority of the cases was diagnosed in the spring. Preceding infection, usually associated with fever and localized in the upper respiratory tract, was the most common cause. The majority of the congenital cases was hereditary and associated with other malformations involving in particular the immune system. In about half of the cases the course was spontaneous and no treatment was necessary; the remaining patients were treated with glucocorticoids, including 46 patients, who had undergone splenectomy. 22 patients died, including 16 cases due to serious underlying disorders; in 6 cases the cause of death was haemorrhage (mortality rat=1.4%). At the follow up the platelet count was below 50 mia/1 in 14 patients (4%). No difference was found concerning the platelet count at the follow up between the patients with spontaneous recovery, the patients treated with glucocorticoids and the patients, in whom splenectomy had been performed. During the course the disease autoimmune haemolytic anaemia occurred in 2 patients, whereas no patient developed systemic lupus erythematosus.     

Study of Leukopenias and Thrombocytopenias by the Direct Antiglobulin Consumption Test on Leukocytes and/or Platelets

A new test, the direct antiglobulin consumption test (direct ACT), has beendevised to be performed on the leukocytes and platelets of patients. It wasperformed in parallel with the indirect antiglobulin consumption test (indirectACT), and other serologic tests on 492 individuals comprising 24 cases of diffuse lupus erythematosus, 93 primary thrombocytopenias, 18 secondarythrombocytopenias, 48 primary leukopenias or leukothrombocytopenias, 80secondary leukopenias, 101 non-leukothrombocytopenic patients and 128 normal subjects.

A good correlation was obtained between a positive or negative result andthe presence or absence of a cytopenia in the corresponding cell series. Outof 183 thrombocytopenic patients, 42.2 per cent gave a positive result withplatelets, and out of 128 leukopenic patients, 25.8 per cent gave a positive result on leukocytes, whereas of the 299 patients with normal leukocyte andplatelet counts, 98 per cent gave negative results. The test was found positivein three categories of patients:

(1) In diffuse lupus erythematosus, tests on both leukocytes and plateletswere almost uniformly positive. The indirect ACT permits a distinctionto be made between three substances in the serum of these patients.These are antiplatelet, antileukocyte-cytoplasm and antileukocyte-nuclearsubstances.

(2) The thrombocytopenic patients were subdivided into two groups, namely primary and secondary thrombocytopenia:

(a) Out of 93 cases of idiopathic thrombocytopenic purpura, about50 per cent gave a positive result with platelets. Neither thehistory nor the clinical picture suggested any differentiation between those who gave negative results, apart from the fact thata history of infection was more frequent among the negativecases. Corticosteroid therapy did not affect the result of the test,but following splenectomy, 38 per cent of the cases become negative.

(b) Of 18 patients with a secondary thrombocytopenia, three cases(16 per cent) gave a positive result.

(3) The leukopenic and leukothrombocytopenic patients were also subdividedinto two groups:

(a) Out of 48 primary or idiopathic cases, some 50 per cent gave apositive direct ACT either on leukocytes and/or on platelets. Byusing the indirect ACT it was possible to distinguish two substances in the serum, one being antiplatelet and the other an antileukocyte cytoplasmic substance.

(b) The 80 cases of secondary leukopenia or leukothrombocytopeniagave a positive result in 15 per cent of cases with leukocytes and/or with platelets.

Of the 101 non-leukothrombocytopenic patients, only five were found togive positive tests.

All the 128 normal subjects gave negative results.

The direct ACT provides direct evidence of the presence of a -globulin,probably an auto-antibody, on the leukocytes and/or the platelets of some 99per cent of cases of diffuse lupus erythematosus, of about 50 per cent ofcases of idiopathic thrombocytopenia and idiopathic leukothromobcytopenia,and in about 15 per cent of cases of secondary thrombocytopenia and leukothrombocytopenia. No marked difference was found in the history, clinicalpicture, or hematological findings between patients giving positive and negative results.

Submitted on April 3, 1961 Accepted on August 1, 1961     

Splenectomy for thrombocytopenia associated with systemic lupus erythematosus in 11 Chinese patients

Since the role of splenectomy in treating thrombocytopenia associated with systemic lupus erythematosus has been controversial, the study was focused on determining the efficacy of splenectomy in the SLE-associated thrombocytopenia. Between 1980 and 2008, 11 patients with SLE underwent splenectomy for treating their thrombocytopenia. Surgical indications, operative mortality and morbidity, and haematological outcomes were followed in both short term (first 30 days) and long term (last recorded platelet count, last contact, or death). Indications for splenectomy included: thrombocytopenia refractory to (63.7%), dependent on (27.3%), or patient intolerance of (9%) medical treatments. Perioperative mortality and morbidity was 0%. The overall rate of early partial or complete response rate to splenectomy was 100%. After a median follow-up of 36 months, 9 (81.9%) patients had sustained complete or partial response without relapse. Eight (72.8%) of these patients required adjunctive medical therapy, whereas the other 1 (9%) did not. The remaining 2 (18.2%) patients relapsed, could not been subsequently salvaged to at least partial response with further treatments. The overall PR or CR to splenectomy combined with medical therapy was 81.8%. Splenectomy should be considered as a safe and efficacious therapy for the severe thrombocytopenia associated with SLE in some selected patients.     

Efficacy and safety of laparoscopic splenectomy in thrombocytopenia secondary to systemic lupus erythematosus

This study aims to investigate the efficacy and safety of laparoscopic splenectomy (LS) in the management of refractory thrombocytopenia associated with systemic lupus erythematosus (SLE). From January 2003 to February 2012, 20 patients underwent splenectomy for thrombocytopenia associated with SLE. Of these, 11 underwent open (SLE-OS group) and 9 underwent laparoscopic splenectomy (SLE-LS group). Another 15 patients with ITP underwent LS (ITP-LS group) were categorized as the control group. Surgical indications, perioperative details, and short- (90 days) and long- (median, 42 months) term hematological outcomes were assessed. Splenectomy was successful in all 20 SLE patients. The mean platelet count increased from 23?×?10⁹/L before splenectomy to 289.2?×?10⁹/L and 144.2?×?10⁹/L after 3 and 6 months, respectively, and was 115.5?×?10⁹/L at the last visit, with a 3-month complete response (CR) rate of 100 %. After a median follow-up of 42 months, 17 patients (85 %) had a CR or partial response (PR) to splenectomy plus medical therapy. SLEDAI score and dosage of steroids decreased significantly after splenectomy. None of these patients experienced any postoperative infection, bleeding, or thrombotic events. SLE-LS group had lower volumes of estimated blood loss and postoperative drainage and shorter postoperative hospital stay than SLE-OS group. There were no statistically significant differences between the SLE-LS and ITP-LS groups in operation time, estimated blood loss, and postoperative hospital stay. Splenectomy is effective and safe in the management of refractory thrombocytopenia secondary to SLE. LS may be safe and effective in thrombocytopenia associated with SLE.     

Splenectomy does not cure the thrombocytopenia of systemic lupus erythematosus

Fourteen patients with systemic lupus erythematosus had splenectomies done between 1960 and 1982 for treatment of severe thrombocytopenia. Thrombocytopenia persisted or recurred within 1 month postoperatively in five patients and within 6 months in three others. Three patients had late recurrence (18, 30, and 54 months after splenectomy); in two it was probably related to withdrawal of immunosuppressive agents or corticosteroids. Median lowest platelet count before splenectomy and median platelet count at relapse or failure of splenectomy were both 8000/microL. Only two patients maintained normal platelet counts without need for corticosteroids or other treatment. These results differ from those in patients with idiopathic thrombocytopenic purpura. Other treatments should be tried before splenectomy is done for thrombocytopenia in patients with systemic lupus erythematosus.     

Plasma Exchange for Thrombocytopenia in Antiphospholipid Syndrome: A Case Report

Abstract: The remarkable effect of plasma exchange (PE) was observed on thrombocytopenia in a patient with antiphospholipid syndrome (APS) associated with systemic lupus erythematosus. Three times PE led to recovery from severe thrombocytopenia refractory to treatment with corticosteroid, anticoagulant, and antiplatelet drugs accompanied by a decrease in the serum cardiolipin β₂ glycoprotein I antibody level. This result suggests that PE is a valuable therapeutic tool for refractory thrombocytopenia in APS.     

Thrombocytopenia in SLE and related autoimmune disorders: association with anticardiolipin antibody

Anticardiolipin antibody levels were determined in 116 patients with systemic lupus erythematosus and related autoimmune disorders. Forty-three of these patients had a history of thrombocytopenia—36 of whom had SLE, three primary Sjögren's syndrome, two rheumatoid arthritis and two mixed connective tissue disease. IgG anticardiolipin antibody levels were raised in 31 (72%) of the 43 patients and IgM anticardiolipin antibody levels were raised in 19 (44%). There was a strong statistical correlation between thrombocytopenia and raised anticardiolipin antibody levels of both the IgG (P < 0.001) and IgM (P < 0.01) immunoglobulin classes. Of the 20 patients with the highest IgG anticardiolipin antibody levels 16 had a history of thrombocytopenia. We suggest that anticardiolipin antibodies may play a direct role in mediating platelet destruction in autoimmune disorders.     

Thrombocytopenia following sustained-release procainamide

Procainamide hydrochloride-induced thrombocytopenia has infrequently been reported in the past. We report six cases of thrombocytopenia following the administration of the sustained-release form of procainamide. Three of these cases had platelet counts of less than 15,000/microL. The mean time to onset of thrombocytopenia from drug administration was 40 days (range, nine to 71 days). The mean time until normalization of the platelet counts after the drug therapy was stopped was 7.8 +/- 3.1 days (range, four to nine days). Oral prednisone therapy had little apparent benefit. The thrombocytopenia was not part of a systemic lupus erythematosus syndrome. We believe that thrombocytopenia is an important side effect of sustained-release procainamide therapy.     

Danazol for the treatment of refractory autoimmune thrombocytopenia in systemic lupus erythematosus

STUDY OBJECTIVE: to determine the efficacy of danazol therapy in patients with systemic lupus erythematosus with severe autoimmune thrombocytopenia refractory to other therapies. DESIGN: noncontrolled clinical trial, with a minimum of 8 weeks of therapy, the maximum determined by clinical response. SETTING: referral-based rheumatology clinic at an army medical center. PATIENTS: sequential sample of six patients with systemic lupus erythematosus with severe autoimmune thrombocytopenia refractory to high-dose glucocorticoids. Four patients also failed splenectomy, or cytotoxic drugs, or both. INTERVENTIONS: danazol, 200 mg four times per day, was added to the previous therapeutic regimen for at least 2 months. MEASUREMENTS AND MAIN RESULTS: all six patients had normal platelet counts within 6 weeks of starting danazol treatment. After resolution of thrombocytopenia for at least 1 month, immunosuppressive medications were tapered; one patient had a relapse. During an average follow-up of 12 months, the danazol dose was lowered in the five remaining patients but could not be discontinued without recurrence of thrombocytopenia. During danazol therapy, platelet-bound IgG antibodies and circulating immune complexes did not decrease significantly. Danazol was well tolerated. CONCLUSIONS: danazol appears to be a useful adjunctive treatment for refractory autoimmune thrombocytopenia associated with systemic lupus erythematosus.     

Prevalence,patterns of disease and outcome in patients with systemic lupus erythematosus who develop severe haematological problems

OBJECTIVE: To evaluate the prevalence of major haemolytic disease-severe autoimmune haemolytic anaemia and severe thrombocytopenia-and to assess when these features develop. We also sought to analyse the clinical and serological outcomes of patients with haemolytic anaemia and thrombocytopenia with systemic lupus erythematosus (SLE) as compared with patients without these cytopenias. METHODS: We reviewed retrospectively all the available case notes from our lupus cohort of 305 patients followed up between 1978 and 2000 (mean follow-up 7 yr). We identified 30 patients with SLE (9.8%), of whom 20 (6.6%) had severe haemolytic anaemia and 10 (3.3%) had severe thrombocytopenia. Each patient was matched for age, sex and ethnicity with two control patients. RESULTS: We recorded a total of 42 episodes of severe haematological events: four patients had a second haemolytic episode and eight patients had a second thrombocytopenic episode. Five patients had both thrombocytopenia and haemolytic anaemia. One per cent of patients had severe haemolytic anaemia prior to the diagnosis of SLE and 2.5% of patients presented with these haematological disorders. Haemolytic anaemia and thrombocytopenia were associated with renal involvement (0.01>P>0.001) and anticardiolipin antibodies (ACL) (0.01>P>0.001), but not anti-dsDNA antibodies. Calculation of the BILAG index at the time of severe haematological crisis demonstrated that renal, central nervous system involvement and general symptoms are more frequently present. Forty-one per cent of patients were already on either prednisolone (<10 mg) or an immunosuppressive agent at the onset of the event. CONCLUSION: Our data demonstrate that both haemolytic anaemia and thrombocytopenia are associated with ACL but not anti-dsDNA antibodies. When faced with a patient with a severe haematological manifestation of lupus, active disease in other organs is likely to be present.     

Intermittent cyclophosphamide for the treatment of autoimmune thrombocytopenia in systemic lupus erythematosus

STUDY OBJECTIVE: To determine the effect of monthly intravenous cyclophosphamide therapy in patients with systemic lupus erythematosus and autoimmune thrombocytopenia. DESIGN: Uncontrolled, retrospective clinical study. SETTING: Government referral-based research hospital. PATIENTS: Seven patients with systemic lupus erythematosus and 2 or more months of thrombocytopenia refractory to or requiring excessive doses of corticosteroids. Two patients had also failed to respond to splenectomy and repeated intravenous methylprednisolone infusions. Six patients had severe active renal disease at the time of treatment. INTERVENTIONS: Cyclophosphamide, 0.75 to 1.0 g/m2 body surface area, was given intravenously every month for at least 4 months. Prednisone dose ranged between 0.5 to 1.0 mg/kg.d. MEASUREMENTS AND MAIN RESULTS: All seven patients had normal platelet counts within 2 to 18 weeks after cyclophosphamide treatment (one to four doses). Prednisone was tapered to 0.25 mg/kg on alternate days in all patients. All six patients had significant improvement in their renal disease and lupus serologies. Cyclophosphamide was discontinued after four to six doses in five patients. Four patients maintained normal platelet counts on low dose, alternate-day prednisone for a mean of 5.6 years of follow-up. Two patients had recurrence of thrombocytopenia 1 to 3 years after discontinuing cyclophosphamide. CONCLUSIONS: Monthly intravenous cyclophosphamide is potentially useful for the management of autoimmune thrombocytopenia in patients with systemic lupus erythematosus who are refractory to or dependent on unacceptably high doses of corticosteroids, or are experiencing side effects of conventional medical or surgical treatment.     

Thrombocytopenia in patients with systemic lupus erythematosus: significant in the clinical implication and prognosis

The purpose of this investigation was to determine the characteristics of the clinical manifestations, laboratory findings and prognostic impact of the thrombocytopenia patients with systemic lupus erythematosus (SLE). The case group of 47 SLE patients with thrombocytopenia and the control of 49 SLE patients with normal platelet number were reviewed retrospectively. The clinical manifestations, immunological profiles, hemograms and myelograms, disease activity (SLEDAI-2K), end organ damages (SLICC) and survival rate at the time of follow-up were recorded. The analysis of clinical manifestations showed that the case group was more likely to be with renal disease (p = 0.038), and less skin rash (p = 0.032). The analysis of the hemograms revealed that the case group was more concomitant with anemia, leucopenia and neutropenia than the control group (p < 0.001, p = 0.014 and p = 0.02, respectively). Moreover, it was also revealed that the SLE patients with thrombocytopenia were often in the condition of higher disease activity, had more probability of end organ damage, and were associated with a higher mortality rate (P = 0.041, p = 0.035 and p = 0.038, respectively). The clinical data and laboratory findings were different between the SLE patients with thrombocytopenia and those with normal platelet number. Thrombocytopenia can be recognized as a reliable prognostic marker to identify a subset of patients with higher disease activity, more possibility of end organ damage and higher mortality.     

Thrombocytopenia as a Prognostic Marker for Systemic Lupus Erythematosus: A Systematic Review and Meta-analysis

BackgroundSystemic lupus erythematosus (SLE) patients often exhibit hematological abnormalities, but the role of thrombocytopenia on the prognosis of SLE shows inconsistent results. The purpose of this meta-analysis was to confirm the impact of thrombocytopenia on mortality and end organ damage in patients with SLE.Materials and MethodsThree electronic databases, PubMed, Embase and Cochrane library were systematically searched to identify the eligible studies from inception to November 2017 in order to evaluate the impact of thrombocytopenia on the prognosis of patients with SLE. The summary odds ratios (ORs) and 95% confidence intervals (CIs) were used to measure the impact of thrombocytopenia on mortality and end organ damage based on the random-effects model.ResultsA total of 8 studies that reported data on 2,158 patients with SLE were included. The summary OR indicated that SLE subjects with thrombocytopenia were significantly associated with an increased risk of mortality (OR: 4.57; 95% CI: 2.28-9.17; P < 0.001) and end-organ damage (OR: 3.31; 95% CI: 1.11-9.86; P = 0.031). Furthermore, the sensitivity analysis indicated stable mortality, while the result for end organ damage was variable. In addition, the patients with thrombocytopenia with disease duration <60 months presented a greater risk for mortality than those with disease duration ≥60.0 months (P = 0.002).ConclusionsPatients with SLE and thrombocytopenia were found to be associated with an increased risk of mortality and end organ damage.     

Thrombotic risk in patients submitted to splenectomy for systemic lupus erythematosus and antiphospholipid antibody syndrome-related thrombocytopenia

Background: Splenectomy has been performed to treat refractory autoimmune thrombocytopenia. However, some reports have suggested that an increased risk of thrombosis could be present in splenectomized patients. This study aims to evaluate the possibility of an increased risk of thrombosis after splenectomy in patients with systemic lupus and antiphospholipid syndrome. Methods: Thrombotic-related events in patients with systemic lupus erythematosus (SLE) and/or primary antiphospholipid syndrome (PAPS), before and after splenectomy for severe thrombocytopenia, were compared. Clinical data, laboratory investigations, and anticoagulation or antiaggregation treatment data were collected from the notes of outpatients attending three European centers. Results: Twenty patients who had had a splenectomy were identified: eight with SLE, five with PAPS, and seven with SLE and APS. The mean time between diagnosis and splenectomy was 3.1 years and mean follow-up was 6.5 years. There were no differences in anticardiolipin antibody titers, lupus anticoagulant, anti-DNA or anti-nuclear antibodies before and after surgery. The incidence of venous events before and after splenectomy was not significantly different. There was a trend towards an increase in the total number of arterial events post-splenectomy. In aCL-positive patients, and in the pre-splenectomy period, the total number of miscarriages was higher (p=0.017), as was the number of patients who had had a miscarriage (p=0.025). Conclusions: The total risk of thrombosis in patients with PAPS and SLE was not increased after splenectomy, but there was a trend towards an increase in the number of arterial events. Splenectomy induced long-term remission of thrombocytopenia (partial or complete) in all patients.     

Thrombocytopenia in homosexual men

The office records of two private practices with a preponderance of homosexual patients were reviewed for cases of males with unexplained thrombocytopenia. Twenty-five patients meeting the selection criteria were found. The clinical and laboratory features of these patients were reviewed and compared to those characteristically seen in classic autoimmune thrombocytopenic purpura. The mean age of the group was 36.5 years. There was a high incidence of a history of sexually transmitted diseases. Sixty percent had another hematologic abnormality in addition to thrombocytopenia. The clinical outcomes for these 25 patients were as follows: eight (32%) had a spontaneous increase in platelets; four (16%) had a stable count not requiring therapy; 12 (48%) received high-dose prednisone; seven (28%) failed prednisone therapy and went on to splenectomy; two (8%) subsequently developed CDC-defined AIDS.     

Thrombocytopenia in homosexual patients. Prognosis, response to therapy, and prevalence of antibody to the retrovirus associated with the acquired immunodeficiency syndrome

Thirty-three homosexual patients with thrombocytopenia (mean [+/- SE] platelet count, 50 000 +/- 7000/mm3; range, 7 to 135 000/mm3) have been followed for a mean period of 20 +/- 2 months. Six patients have developed the acquired immunodeficiency syndrome 1 to 37 months after the diagnosis of thrombocytopenia. Six patients spontaneously reverted to normal platelet counts 5 to 27 months (median, 10 months) after the diagnosis of thrombocytopenia, in the absence of splenectomy and while not receiving corticosteroids. Sixteen of seventeen patients had a moderate to excellent response while on corticosteroid treatment. Ten of ten patients had an excellent response to splenectomy which has persisted. Fifteen patients did not require treatment for their thrombocytopenia. Thirteen of fourteen patients had antibody against the retrovirus associated with the acquired immunodeficiency syndrome, as did 4 of 12 homosexual controls without thrombocytopenia. Thrombocytopenia in homosexuals is part of the complex related to the acquired immunodeficiency syndrome.     

Neonatal Outcomes of Pregnancy with Immune Thrombocytopenia

Neonates born to mothers with immune thrombocytopenia (ITP) have an increased risk for neonatal thrombocytopenia and hemorrhagic complications. The aim of this study was to determine the maternal and neonatal outcomes of pregnancies with ITP and also to identify risk factors that predicts neonatal thrombocytopenia. We performed a retrospective analysis of 40 pregnancies with ITP and their 40 neonates. Among the 40 neonates, thrombocytopenia (platelet count of less than 150 × 10⁹/L) was detected in 15 neonates (37.5 %) whom 8 of them had severe thrombocytopenia (platelet count of less than 50 × 10⁹/L). Ten of the 15 neonates with thrombocytopenia required treatment to increase the platelet counts. There was statistically significant association between neonatal thrombocytopenia and maternal splenectomy history and maternal duration of thrombocytopenia. There was no statistically significant correlation between maternal platelet count and neonatal platelet count. Clinicians should pay special attention in these neonates because of risk for development of neonatal thrombocytopenia. Maternal and neonatal outcomes in patients with idiopathic thrombocytopenic purpura is generally good.     

Idiopathic portal hypertension associated with systemic lupus erythematosus

A case of idiopathic portal hypertension (IPH) associated with systemic lupus erythematosus (SLE) is reported in a 38-year-old man who had been diagnosed with SLE and treated for 18 years. Esophageal varices, found in 1994 on endoscopic examination, had been followed up for 2 years. On July 16, 1996, he was admitted to Nagoya University Hospital because there was a high risk of bleeding from the esophageal varices due to severe thrombocytopenia. As partial splenic embolization had temporarily controlled the thrombocytopenia, splenectomy and devascularization of the stomach vessels were performed after endoscopic ligation of the esophageal varices. Histological specimens of wedge biopsied liver showed chronic inactive hepatitis without cirrhosis. The presence of anticardiolipin antibody, indicated by positivity for lupus anticoagulant, was suggestive of the presence of a common immunological mechanism in the etiology of SLE and IPH. Received: January 20, 1999 / Accepted: July 23, 1999     

Prevalence of anticardiolipin antibodies in subacute cutaneous lupus erythematosus.

We examined the prevalence of the antibodies to cardiolipin measured by solid-phase enzyme immunoassay during a prospective study of patients with subacute cutaneous lupus erythematosus (SCLE). Seven of 44 (16%) consecutive patients with SCLE had positive anticardiolipin antibodies; of these only three satisfied the American Rheumatism Association's revised criteria for the classification of systemic lupus erythematosus. Clinical findings probably associated with the positive anticardiolipin antibodies were found in four cases, including clotting abnormalities, thrombocytopenia, hemolytic anemia, livedo reticularis, chilblain lupus erythematosus lesions, migraine, leg venous thrombosis and pulmonary embolism after surgery, and spontaneous abortion. Our data suggest that it is reasonable to screen SCLE patients for these antibodies to confirm the presence of the antiphospholipid syndrome.