Successful treatment of subdural hemorrhage and retinal hemorrhage in childhood-onset systemic lupus erythematosus associated with thrombocytopenia: Case report

Introduction:Thrombocytopenia (TP) is a common complication of childhood-onset systemic lupus erythematosus (SLE), and can range from mild to life-threatening. However, severe TP with multiple hemorrhagic complications is very rare and often predicts a poor prognosis. We describe a 12-year-old Chinese girl who had a history of idiopathic thrombocytopenic purpura who developed SLE that presented as subdural hemorrhage and retinal hemorrhage because of severe TP.Patient concerns:A 12-year-old girl was admitted into our hospital because of fever, purpura, and gum bleeding lasting for 12 days. She had a history of idiopathic thrombocytopenic purpura 2 years ago previously.Diagnosis:SLE was diagnosed according to American College of Rheumatology classification criteria. Subdural hemorrhage and retinal hemorrhage were diagnosed based on brain MRI and funduscopy. Severe TP was defined as platelet count <20 × 10⁹/L.Interventions:She was treated first with intravenous immunoglobulin, but it was not efficacious. High-dose methylprednisolone showed short-term efficacy. Then, she was given a glucocorticoid and cyclosporine A plus mycophenolate mofetil.Outcomes:Fever, purpura, and gum bleeding were resolved before hospital discharge. Subdural hemorrhage and left hemorrhagic retinopathy were improved remarkably. She had a durable response to refractory TP with no adverse effects during >1-year follow-up.Conclusion:Isolated TP may be an early symptom of childhood-onset SLE . A child with severe TP is prone to develop life-threatening hemorrhagic complications. Glucocorticoids and combined immunosuppressive drugs had a durable response to refractory TP in this patient with no adverse effects.     

Thromboembolism after cardiac surgery in a patient with cancer-related thrombosis and severe thrombocytopenia: A case report

Rationale:Patients with cancer have elevated risk of both venous thromboembolism and bleeding compared with patients without cancer due to cancer- and patient-specific factors. Balancing the increased and competing risks of clotting and bleeding in these patients can be difficult because management of cancer-associated thrombosis requires anticoagulation despite its known increased risks for bleeding. The adjustment of blood transfusion or cessation of anticoagulants can be a challenge in surgical diagnosis or treatment of cancer patients with such an imbalanced coagulate status.Patient concerns:A 45-year-old woman with no underlying disease was suspected of ovarian cancer and was awaiting diagnostic laparoscopic exploration surgery.Diagnoses:While waiting for the surgery, the patient developed chest pain and underwent stent insertion under diagnosis of myocardial infarction. Two weeks later, endocarditis developed, and replacement of the aortic valve and mitral valve was planned. In addition, the patient developed multiple thromboembolisms and was administered anticoagulants to eliminate vegetation of valves and multiple thromboses. Her blood test showed anemia (7.4 g/dL) and severe thrombocytopenia (24 × 10⁹/L).Interventions:The patient underwent double valve replacement.Outcomes:A color change of the left lower extremity was noted 5 hours after double valve replacement, and angiography was performed. Thrombectomy was performed under diagnosis of thrombosis in the left iliac artery. One month later, the patient underwent laparoscopic exploration surgery as scheduled.Lessons:This case will help establish the criteria of blood coagulation for surgical treatment of cancer patients with imbalanced clotting and bleeding.     

Scrub typhus: Clinical spectrum and outcome

Background:

Scrub typhus is one of the differential diagnoses for fever with thrombocytopenia. ARDS associated with Scrub typhus has high morbidity and mortality.

Aims:

To evaluate clinical features, lab values, and outcome in patients with scrub typhus and comparison in patients with or without ARDS.

Methods:

A prospective observational study was conducted on 109 patients with febrile illness and thrombocytopenia during a period of 12 months. All 109 patients were tested with both Immune-chromatography test and Weil felix test. Patients having either Immune-chromatography test/Weil felix test positive have been included and considered as scrub typhus positive whereas negative for both Immune-chromatography and Weil felix test were excluded. Clinical features, lab parameters, and outcome were evaluated in all patients with scrub typhus. Statistical analysis used in this study was T-test.

Results:

Among 58 patients who were included (After exclusion of 51 patients among total of 109 patients) 34 patients had no ARDS and 24 patients had ARDS. The clinical feature like dyspnoea, cough, low blood pressure (MAP<65 mmHg), IVC collapsibility (by ultrasound) and laboratory parameters like decreased Hemoglobin, Hematocrit, Serum albumin, and increased serum creatinine, serum total bilirubin, SGOT, SGPT, LDH, CPK, and serum lactate were statistically significant (P < 0.0001) in scrub typhus patients group with ARDS. The higher titers of Weil-felix can be correlated with more severe form of disease according to our observation. All 34 Scrub typhus patients without ARDS recovered completely. Among 24 Scrub typhus patients with ARDS, 22 patients recovered, and 2 patients died.

Conclusion:

Scrub typhus is an important differential diagnosis in a patients having fever with thrombocytopenia. Scrub typhus associated with ARDS has high morbidity and mortality. Early diagnosis and treatment with doxycycline can prevent the occurrence of ARDS     

Type IIB von Willebrand's disease associated with a complex thrombocytopenic thrombocytopathy

A familial bleeding disorder characterized by an association of Type IIB von Willebrand's disease (vWD) with a complex thrombocytopenic thrombocytopathy is described in two patients from the same generation. Findings typical of type IIB vWD included enhanced ristocetin-induced binding of patient von Willebrand factor (vWF) to platelets of patients and normal individuals in association with the absence of larger multimers from plasma. Abnormalities in platelet function included deficient platelet aggregation to ADP, collagen, epinephrine, and arachidonic acid; and defective release of 14C-serotonin, vWF, and platelet factor 4 (PF4) in response to thrombin, collagen, or ADP. Platelet factor 4 and platelet vWF were decreased when measured per mg of total platelet protein. In addition, the binding of normal vWF to patient platelets stimulated with thrombin was decreased. Platelet size was increased with a very heterogeneous distribution width. Electron microscopic evaluation showed giant platelets with dense and alpha bodies present. The platelet count was borderline or slightly decreased in the resting state and declined to frankly thrombocytopenic levels at the time of acute bleeding episodes; this state was associated with the presence of platelet aggregates in blood smears.     

Targeted-immunosuppression with vincristine infusion in the treatment of immune thrombocytopenia

Abstract Twenty-four patients with immune thrombocytopenia (ITP) were treated with vincristine (VCR) 1.0–2.0mg given as 4-hr I.V. infusions at weekly intervals for four-six weeks; four patients received further infusions, as maintenance therapy, at increasing intervals for up to 12 months. Eight often patients with recent-onset (< 6 months) ITP showed an excellent and sustained response, 7/8 without maintenance therapy. Among the 14 patients with ITP of >6 months' duration, seven showed a good or excellent but only transient response; sustained responses (two good, one partial) were seen only in the three patients who received maintenance therapy. The collective global experience with this novel therapeutic approach of targeted-immunosuppression for ITP is still small, but results to date suggest a promising role for this approach, especially in patients with recent-onset ITP. (Aust NZ J Med 1991; 21: 405–407.)     

Steroid pulse therapy in patients with encephalopathy associated with severe fever with thrombocytopenia syndrome

Severe fever with thrombocytopenia syndrome (SFTS) is a tick-borne infectious disease caused by the SFTS virus (SFTSV). Clinical symptoms of SFTS often involve encephalopathy and other central neurological symptoms, particularly in seriously ill patients; however, pathogenesis of encephalopathy by SFTSV is largely unknown. Herein, we present case reports of three patients with SFTS, complicated by encephalopathy, admitted to Tokushima University hospital: one patient was a 63-year-old man, while the other two were 83- and 86-year-old women. All of them developed disturbance of consciousness around the 7th day post onset of fever. After methylprednisolone pulse therapy of 500 mg/day, all of them recovered without any neurological sequelae. SFTSV genome was not detected in the cerebrospinal fluid of 2 out of the 3 patients that were available for examination. In these patients, disturbance of consciousness seemed to be an indirect effect of the cytokine storm triggered by SFTSV infection. We propose that short-term glucocorticoid therapy might be beneficial in the treatment of encephalopathy during early phase of SFTSV infection.     

Thrombocytopenia in high‐risk patients with antiphospholipid syndrome

Essentials

• The prevalence of thrombocytopenia in patients with antiphospholipid syndrome is not well defined.
• We studied triple positive patients with antiphospholipid syndrome and its catastrophic variant.
• Prevalence of thrombocytopenia was 6% and 100% in patients who developed the catastrophic form.
• In triple positive patients thrombocytopenia is low and platelets drop during the catastrophic form.

Summary

Background

Thrombocytopenia is the most common non‐criteria hematological feature in patients with antiphospholipid syndrome (APS). This condition is more common in patients with catastrophic APS (CAPS).

Objectives

To evaluate the prevalence of thrombocytopenia in a large series of high‐risk patients with APS, and to assess the behavior of the platelet count during CAPS.

Methods/Patients

This was a cross‐sectional study in which we analyzed the platelet counts of a homogeneous group of high‐risk APS patients (triple‐positive). Six of these patients developed a catastrophic phase of the disease, and the platelet count was recorded before the acute phase, during the acute phase, and at recovery.

Results

The mean platelet count in 119 high‐risk triple‐positive patients was 210 × 10⁹ L^?1. With a cut‐off value for thrombocytopenia of 100 × 10⁹ L^?1, the prevalence of thrombocytopenia was 6% (seven patients). No difference between primary APS and secondary APS was found. In patients who suffered from CAPS, a significant decrease from the basal count (212 ± 51 × 10⁹ L^?1) to that at the time of diagnosis (60 ± 33 × 10⁹ L^?1) was observed. The platelet count became normal again at the time of complete remission (220 ± 57 × 10⁹ L^?1). A decrease in platelet count always preceded the full clinical picture.

Conclusions

This study shows that, in high‐risk APS patients, the prevalence of thrombocytopenia is low. A decrease in platelet count was observed in all of the patients who developed the catastrophic form of the disease. A decrease in platelet count in high‐risk APS patients should be considered a warning signal for disease progression to CAPS.