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Maternal hemolysis, elevated liver enzymes, low platelet count, and neonatal outcome.

Pregnancies complicated by the syndrome of hemolysis, elevated liver enzymes, and low platelet count (HELLP) have been associated with both a poor maternal and a poor neonatal outcome in several publications. Because many studies were small and gave only scant information regarding the infants, we analyzed the clinical course of 89 neonates born to mothers with the HELLP syndrome. Ninety-eight percent of the neonates were born by cesarean section. Infants with a maternal HELLP syndrome were often small for gestational age (39%). The incidence of perinatal asphyxia was found to be 5.6%. Additionally, the affected very low birthweight (VLBW) infants had relatively high incidences of leukopenia (21%), neutropenia (33%), and thrombocytopenia (33%). Initially, 54% of the LBW infants were found to have normoblasts and 25% erythrocytosis. The incidence of these changes in blood cell count increased with decreasing birthweight. Nosocomial infections occurred more often in infants with a reduced neutrophil count. The overall mortality rate was 56 per 1000. Comparing the statistics of the VLBW infants with a maternal HELLP syndrome (n = 32) to all infants with a birthweight less than 1500 g (n = 521) during the investigational period, we found a similar mortality rate (9.3% and 8.4%, respectively). The pulmonary morbidity was also similar. The incidence of intracranial hemorrhage in VLBW infants with a maternal HELLP syndrome was lower (12.5% versus 18.2%) and of necrotizing enterocolitis was higher (6.2% versus 1.9%).

Birth Weight↗

Hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome with acute cortical blindness.

The coincidence of hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome and cortical blindness is an uncommon but very dramatic event. We describe a case of HELLP syndrome complicating with acute cortical blindness before delivery. A 27 year-old woman, gravida 1, para 0, with normal medical history, was referred to our emergency department at the 33th week of gestation due to headache, vomiting, and blurred vision. The ophthalmologic examination showed intact pupillary light reflexes and normal ophthalmoscopic findings, but no light perception in either eye. Brain computed tomography showed normal findings. HELLP syndrome and preeclampsia was diagnosed based on the findings of hypertension and proteinuria as well as laboratory data. Prompt delivery was performed in order to achieve good maternal and neonatal outcomes.

Acute Disease↗

Garcia's disease. Cyclic thrombocytopenic purpura in a child and abnormal platelet counts in his family.

After a critical study, a splenectomy was performed in a 6-year-old boy with chronic thrombocytopenic purpura. Failure of surgery and immunosuppressive therapy prompted new investigations which led to the discovery of a cyclic thrombocytopenic purpura related to a periodic variation in maturity of megakaryocytes. The patient's platelets were morphologically and functionally normal and it was not possible to demonstrate neither immunological mechanism, nutritional deficiency, influence of the environment nor consumption or excessive destruction of platelets. Cyclic thrombocytopenia was detected in the father and also cyclic variations in platelet counts from normal values to over 1,000 x 10(9)/1 in 4 of 9 siblings. In view of these findings. The abnormal condition in this family was named Garcia's disease.

Blood Cell Count↗

Peripheral platelet count in response to salbutamol before and after adrenergic beta-receptor blockade.

The effect of salbutamol (a comparatively selective adrenergic beta2-receptor agonist) on the peripheral platelet concentration was studied before and after the ingestion of either 50 mg metoprolol or 40 mg propranolol. The study was carried out on healthy males volunteers and autologous 51Cr-labelled platelets were employed at the experiments. Salbutamol was infused intravenously over a period of 6 min in a dose of 0.27 microgram.kg(-1). min(-1). Prior to metoprolol and propranolol administration a statistically significant lowering in platelet-bound radioactivity (PBR) was obtained in response to the salbutamol infusions. This salbutamol-induced fall in PBR was completely blocked by propranolol but was left unaffected by metoprolol. It is concluded that adrenergic beta2-receptor stimulation induces a transient lowering of the peripheral platelet count.

Adrenergic beta-Antagonists↗

Diabetes insipidus revealing acute myelogenous leukaemia with a high platelet count, monosomy 7 and abnormalities of chromosome 3: a new entity?

We describe three cases of acute myeloid leukaemia revealed by diabetes insipidus. The patients were 42, 38 and 39 yr old and they had marked hyperleukocytosis, circulating immature granular cells and a normal or elevated platelet count. The leukaemia was type AML-M2 according to the FAB classification. Cytogenetic studies showed inversion of chromosome 3 (q21;q26) in 2 cases and a translocation (3;3)(q21;q29?) in the remaining case, both associated with monosomy 7. All the cerebral CT scans were normal. Complete remission was never achieved, and all three patients survived less than 14 months. Desmopressin therapy was active but treatment could not be reduced. The association of dysmegakaryopoiesis with a chromosome 3 abnormality and diabetes insipidus is probably not fortuitous and could represent a new entity.

Adult↗

Bone marrow absorbed dose of rhenium-186-HEDP and the relationship with decreased platelet counts.

UNLABELLED: Rhenium-186(Sn)-1,1-hydroxyethylidene diphosphonate (186Re-HEDP) has been used for palliation of metastatic bone pain. The purpose of this study was to find a relationship between the bone marrow absorbed dose and the toxicity, expressed as the percentage decrease in the peripheral blood platelet count. METHODS: The bone marrow absorbed dose was calculated according to the MIRD model using data obtained from ten treatments of patients suffering from metastatic prostate cancer; noninvasive and pharmacokinetic methods were used. The bone marrow doses were related to toxicity using the pharmacodynamic sigmoid Emax model. RESULTS: The mean bone marrow absorbed doses using the noninvasive and pharmacokinetic methods were in a close range to each other (1.07 mGy/MBq and 1.02 mGy/MBq, respectively). There was a good relationship between the toxicity and the bone marrow absorbed dose (r = 0.80). Furthermore, the EDrm50 (i.e., the bone marrow absorbed dose producing a 50% platelet decrease) to bone marrow for 186Re-HEDP was on the order of 2 Gy. CONCLUSION: Although the function of normal bone marrow is affected by metastases in patients with metastatic bone disease, the MIRD model can be used to relate toxicity to the bone marrow absorbed dose after a therapeutic dosage of 186Re-HEDP.

Aged↗

Fluctuations in the peripheral blood leukocyte and platelet counts in leukocytapheresis in healthy volunteers.

Leukocytapheresis (LCP) for the treatment of patients with diseases that involve an abnormal autoimmune reaction aims to improve the condition of the patient's pathology and to correct imbalances in immunological regulation mechanisms by removing the responsible leukocytes from the peripheral blood. To clarify the mechanism of therapeutic effect, LCP was conducted in healthy volunteers to investigate changes in peripheral blood leukocyte and platelet counts over time during the treatment. The subjects were 10 healthy male volunteers. LCP was performed once in each volunteer for 3,000 ml of blood volume. The peripheral blood counts decreased significantly, reaching a minimum of 20.0% of the baseline number of leukocytes, 10.1% of the baseline number of neutrophils, and 40.3% of the baseline number of lymphocytes. The number of removed leukocytes was about 6.6 x 10(9) cells, including about 3.5 x 10(9) neutrophils, as well as about 5.0 x 10(11) platelets. After the completion of LCP, the peripheral leukocyte levels increased transiently (overshoot), and at 2 h after the completion of the treatment, they reached 193.4% of the baseline value. Since LCP is capable of reducing the peripheral blood leukocyte count over a short period of time, its impact on peripheral blood is great. In addition, in view of the overshoot phenomenon and the appearance of immature granulocytes, the LCP may affect not only the peripheral blood, but also the bone marrow pool, the marginal pool, and the leukocytes present in the tissues.

Adult↗

A dose of 75 microg/kg/d of i.v. anti-D increases the platelet count more rapidly and for a longer period of time than 50 microg/kg/d in adults with immune thrombocytopenic purpura.

Treatment with 75 microg/kg/d intravenous (i.v.) anti-D was compared with 50 microg/kg/d in a prospective randomized study of 27 RhD-positive, human immunodeficiency virus-negative, adult, acute, non-splenectomized patients with immune thrombocytopenic purpura (ITP) and platelet counts < or = 30 x 109/l. The higher dose resulted in greater median d 1 (43 x 109/l vs. 7.5 x 109/l; P = 0.012) and d 7 (153 x 109/l vs. 64.5 x 109/l; P = 0.001) platelet increases despite no greater haemoglobin decrease. Children with acute ITP receiving 75 microg/kg/d had overnight platelet increases in seven out of nine cases. The duration of effect at the 75 microg/kg/d dose was 46 d vs. 21 d (P = 0.03). Adverse events were mild to moderate and ameliorated with prednisone and acetaminophen premedication.

Acetaminophen↗

Responses to single-dose thrombopoietin decrease with higher platelet counts in mice.

Since the description of human thrombopoietin (TPO) we investigated the thrombocytosis-inducing capacity of human serum samples derived from individuals with altered thrombocytopoiesis. Several times the degree of thrombocytosis developing in recipient mice differed markedly even when applying the same human material. In the last 2 years, we applied single doses of recombinant human TPO (rHuTPO) to random-bred CFLP mice, and the same observation was made. Taken together with previous information (before 1970) it was possible to select cases in which the percent increases in circulating platelet counts inversely correlated with the starting levels. It appears, however, that apart from the known absorbing role of platelets and megakaryocytes, the response to single doses of exogenous rHuTPO in mice depends, at least partially, on an unknown endogenous homeostatic mechanism. Mixing thrombopoietically active human sera with platelet-free normal serum in a 1:1 ratio remarkably reduced the thrombocytosis-inducing capacity. Repeated pharmacological doses of TPO, applied in the majority of the reported trials, however, easily obscure the physiological control mechanism.

Animals↗

Discrepancy between antiplatelet antibody activities detected by immunoblot procedure and platelet counts in idiopathic thrombocytopenic purpura.

By immunoblot procedure it is possible to identify the pathogenic autoantibody responsible for platelet destruction in idiopathic thrombocytopenic purpura (ITP). We assessed the relationship between antiplatelet antibody activities detected by this technique and clinical thrombocytopenia in a patient with ITP whose antiplatelet autoantibody was directed toward an 85-kDa antigen. In this patient, over a 2-year-period, the platelet counts were not correlated with the levels of antiplatelet autoantibody detected by immunoblotting. The present observations suggest that IgG autoantibody directed toward a specific antigen is not necessarily a critical determinant of the degree of thrombocytopenia, and that factors other than IgG-Fc-receptor-mediated platelet destruction are also important in the determination of the disease activity in ITP.

Adult↗

Utilization of platelet transfusions in the intensive care unit: indications, transfusion triggers, and platelet count responses.

BACKGROUND: A description of current platelet (PLT) transfusion practice in the intensive care unit (ICU) is needed. STUDY DESIGN AND METHODS: All thrombocytopenic patients (PLT count, <150 x 10(9)/L) who received PLT transfusions were identified from a previous prospective study of consecutive medical-surgical ICU patients; trauma, orthopedic, and cardiac surgery were exclusions. Risk factors for ineffective transfusions were examined. RESULTS: Of 261 ICU patients, 118 (45.2%) had thrombocytopenia and a PLT count nadir of less than 50 x 10(9) per L (n = 22), 50 to 99 x 10(9) per L (n = 37), and 100 to 149 x 10(9) per L (n = 59). Twenty-seven (22.9%) patients received PLT transfusions (n = 76 transfusions) and 37 (31.4%) had major bleeding. PLT dose was approximately 3 to 4 x 10(11) per L transfusion. Therapeutic (n = 24) and prophylactic (n = 52) PLT transfusion triggers were 51 x 10(9) per L (interquartile range [IQR], 26 to 68) and 41 x 10(9) per L (IQR, 20 to 57), respectively, as measured at a median of 4.5 hours (IQR, <1.6 to 6.9) before transfusion. A single PLT transfusion resulted in a median PLT increase of 14 x 10(9) per L (IQR, -2 to 30) measured at 5.2 hours (IQR, 1.8 to 8.8) after the transfusion; however, no PLT count increase was observed after 17 transfusions given to 13 (48.1%) patients. No risk factors for ineffective transfusions were identified. CONCLUSIONS: Among critically ill patients, most PLT transfusions were administered to prevent, rather than to treat, bleeding, with a transfusion trigger of 40 to 50 x 10(9) per L. Nearly half of ICU patients who received transfusions failed to mount a PLT count increase after a single transfusion. Prospective studies are needed to determine the effects of PLT transfusions on bleeding and predictors of ineffective transfusions in the ICU.

Aged↗

A case of myelodysplastic syndrome with high platelet counts and a t(3;8)(q26;q24).

A 62-year-old male patient with refractory anemia with excess of blasts had a t(3;8)(q26;q24) as an acquired chromosomal abnormality in the bone marrow. This aberration has not been previously reported as a sole anomaly in any type of neoplasm, including myelodysplastic or myeloproliferative disorders. The findings of disturbed thrombocytopoiesis with high platelet counts and micromegakaryocytes in the bone marrow support the idea that alteration of the transferrin receptor gene, localized to 3q26, may be of pathogenetic significance.

Anemia, Refractory, with Excess of Blasts↗

Platelet count, antiplatelet therapy and pulmonary embolism--a prospective study in patients with hip surgery.

Pulmonary embolism (PE) is a serious complication following hip surgery. Trials of antiplatelet thromboprophylaxis indicated a substantial reduction in PE rate, and we prospectively studied the effect of a combination of low-dose heparin and two different antiplatelets. Furthermore, our experience in previous studies suggested that platelet count (PC) levels could be useful to reliably suspect PE at a very early stage, and we prospectively tried to confirm our previous findings. Ours is a prospective study in 459 consecutive patients operated on because of hip fracture (265) or elective hip replacement (194), aimed to determine: 1) whether the benefits of antiplatelets plus heparin on PE outweigh the risks; 2) to assess the clinical usefulness of PC monitoring in these patients, so as to confirm whether PE could be recognized early. It was a prospective, randomized, double-blind study. All patients received unfractioned heparin (7500 IU sc twice daily, starting 2 h before operation). In addition, they received aspirin (200 mg thrice daily, with meals), Triflusal (300 mg thrice daily, with meals), or placebo. Real time B-mode ultrasonography (US) was performed on all patients on the 8-9th day after surgery. Venography was performed in patients with normal US, if clinical symptoms suggested venous thrombosis. Twelve out of the 459 patients (2.6%) had to discontinue prophylaxis, because of major bleeding (6 patients), or gastric intolerance (6 patients). There were no significant differences between groups in either deep vein thrombosis (26 patients (18%) with aspirin, 18 (12%) with Triflusal, 26 (17%) with placebo), or PE development (7 patients (5%) with aspirin, 3 (2%) with Triflusal, 8 (5%) taking placebo).(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗

The effect of prophylactic proteinase inhibitor therapy on post-traumatic pulmonary insufficiency and platelet counts.

In a prospective double blind clinical study of 70 patients with bony trauma and shock, 35 patients were allocated into each group receiving either a placebo or Aprotinin. In both groups there was a high incidence of pulmonary insufficiency and thrombocytopaenia. In the placebo group there was a higher incidence of pulmonary insufficiency, but not its severity, with greater volumes of blood transufsion and the most severe form occurred only after decompensated shock. Thrombocytopaenia showed a similar pattern in all patients from both groups falling to a mean minimum on the 2nd day and rising thereafter. In the placebo group counts were lower in those with decompensated shock, and the fall was more profound, rapid and earlier in patients developing severe pulmonary insufficiency. Counts of less than 100,000 on the first day were associated with a high probability of severe pulmonary insufficiency subsequently occurring and this finding was not associated with greater volumes of blood transfusion. In those patients receiving Aprotinin, the subsequent incidence of severity of pulmonary insufficiency was less and not associated with increased volumes of blood transfusion. The platelet counts were not lower in decompensated shock and there was a more rapid rise from the minimum level.

Aprotinin↗

Elevated platelet count features the variant type of BCR/ABL junction in chronic myelogenous leukaemia.

A variant form of BCR/ABL junction was identified in a patient with chronic myelogenous leukaemia (CML). The BCR/ABL fusion mRNA of this patient showed in-frame junction between BCR exon c3 and ABL exon 2. Although the diagnosis of CML was made, the patient showed clinical features of essential thrombocythaemia (ET) rather than that of typical CML. Treatment with interferon-alpha showed no cytogenetic response. The c3-a2 type of BCR/ABL junction seems to be associated with elevated platelet count and thus could form a novel clinical entity different from typical CML.

Adolescent↗