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PubMed2001

Acquired storage-pool disorders occurring late after allogeneic bone marrow transplantation: partial activation of platelets in asymptomatic patients.

Bone marrow transplantation (BMT) may be complicated by coagulation abnormalities. The present study evaluated whether platelets might be activated in patients who had undergone BMT without significant coagulopathy. The patients selected had received allogeneic BMTs a median of 39 months before the study (range, 11-124 months) and had not received cyclosporine, FK506 (tacrolimus), or other medication affecting cyclo-oxygenase for at least 3 months prior to the collection of blood samples. Furthermore, patients had platelet counts greater than 100 x 10(9) cells/L and normal serum creatinine levels. Twenty-five healthy volunteers acted as controls. Platelet aggregation studies and a mepacrine assay of platelets showed abnormal aggregation and decreased staining in some patients. The platelet storage-pool adenosine 5'-triphosphate (ATP) level in 15 patients after BMT was 0.45+/-0.24 micromol per 10(11) platelets, whereas the level in 18 controls was 1.03+/-0.36 micromol per 10(11) platelets (P = .00078). The total ATP levels of platelets in patients and controls were 4.33+/-1.14 and 5.63+/-1.51 micromol per 10(11) platelets, respectively (P = .016). With the exception of 1 patient, plasma levels of thrombomodulin and von Willebrand factor were all within the normal range. The average plasma level of 11-dehydrothromboxane B2 was significantly increased in 15 patients after BMT compared with controls, 20.6+/-8.2 and 10.3+/-1.2 pg/mL, respectively (P = .0004). These findings suggest a long-term process of platelet activation in patients after BMT and, following the cessation of cyclosporine, development of acquired storage-pool disorder of platelets.

Adenosine Triphosphate↗
PubMed2005

delta-Storage pool disease: a pitfall in the forensic investigation of sudden anal blood loss in children: a case report.

We present the case of a 3.5-year-old boy with sudden anal blood loss at school. Sexual abuse was suspected, and, apart from anal fissures seen on sigmoidoscopy, no other clinical signs of any sort of disorder were present. As no medical explanation for the blood loss could be given, penetrating anal trauma was suggested. During follow-up consultations, there were complaints of occasional blood loss. Platelet aggregation tests and electron microscopy finally helped diagnose a delta-storage pool disease which is a rare haemostatic disorder involving the dense granules of the platelets. Although exclusion of well-known blood diseases through routine laboratory testing is a common practice in children with sudden blood loss, this case illustrates the value of more specialised investigation both from a diagnostic and forensic point of view.

Anus Diseases↗
PubMed1987

The capillary thrombometer and von Willebrand factor.

A modified capillary thrombometer was constructed to study the rate of thrombus formation using heparinized whole blood (2 U/ml) from 4 different groups of pigs: normal, heterozygous von Willebrand's disease (vWD), homozygous vWD, and platelet storage pool disease (SPD). The median thrombosis times for the 4 groups of pigs were: 5.3 min (range = 3.0-14), 31 min (range = 4.0-47), 55 min (range = 41-60), and 60 min (range = 15-60), respectively. Significant differences were demonstrated between all pig groups (p less than .01 - p less than .001), except between the homozygous vWD pigs and the SPD pigs (p = 0.8), both of which are clinical bleeders. Cryoprecipitate was infused into 3 pigs with homozygous vWD. Partial correction of the capillary thrombometer thrombosis time and the in vivo ear bleeding time was observed. Murine monoclonal antibodies to porcine von Willebrand factor were added to normal pig whole blood samples in the capillary thrombometer. Four of six antibodies prolonged the thrombosis time and had similar effects on the ear bleeding time. Using these monoclonal antibodies, an immunoperoxidase stain demonstrated plasmatic and platelet associated von Willebrand factor in sections of thrombi from the capillary thrombometer. These experiments confirm that von Willebrand factor is important to thrombus formation in the capillary thrombometer and that measurements by this instrument may relate to in vivo hemostasis as measured by the ear bleeding time.

Animals↗
PubMed1993

Lack of effect of 1-desamino-8-D-arginine vasopressin on direct adhesion of platelets to collagen.

Administration of 1-desamino-8-D-arginine vasopressin (DDAVP), a synthetic vasopressin derivative, causes an increase in plasma factor VIII and von Willebrand factor (vWF). Recently, evidence has become available that intravenous infusion of DDAVP shortens the prolonged bleeding times in some patients with primary platelet defects even though their plasma levels of vWF and FVIII are normal prior to drug administration. The mechanism of this effect of DDAVP has not been well defined and it has been generally considered that the beneficial effect on the bleeding time is related to the rise in plasma vWF and its impact on platelet adhesion to subendothelial components including collagen, an important step in hemostasis. Thus, studies aimed at understanding the effect of DDAVP have focused on vWF-mediated adhesion of platelets to the subendothelium. For example, Sakariassen et al studied the platelet adherence to human arterial subendothelium and concluded that DDAVP improves hemostasis by causing enhanced vWF-mediated platelet adherence. This mechanism would explain the shortening of the bleeding time in patients with milder forms of vWD with subnormal levels of vWF. But patients with congenital platelets defects have normal plasma vWF raising the possibility that there may be other mechanisms contributing to the beneficial effect of DDAVP. It is clear that platelets can interact directly with collagen, mediated by specific platelet binding sites. Further, DDAVP binds to platelets even though by itself does not activate them. The present investigation was designed to elucidate whether DDAVP had any effect on the direct adhesion of platelets to collagen in the absence of mediation by vWF. Hashemi et al have suggested that the effect of DDAVP on endothelial cell release of vWF is mediated by an as yet uncharacterized intermediate factor(s) released from peripheral mononuclear cells. Therefore, we studied the effect also of plasma samples obtained from patients treated with DDAVP on adhesion of platelets to collagen.

Bleeding Time↗
PubMed1989

Early presence of activated ('exhausted') platelets in malignant tumors (breast adenocarcinoma and malignant melanoma).

To evaluate whether or not the finding of platelet activation in patients with tumors is related to the stage of malignancy, a study of biochemical markers indicative of the presence of circulating activated ('exhausted') platelets was carried out in 95 untreated patients with breast adenocarcinoma or malignant melanoma, localized or spread to regional lymph nodes with no detectable distant metastasis. These tumors were chosen as examples of tumors which can be accurately staged for localization or spread, and as examples of mucin-secreting tumors (breast adenocarcinoma) or neuroectodermic tumors (malignant melanoma). Results were compared with those for 26 patients with benign breast disease, 23 blood donors and 50 hospital workers. The most frequent abnormalities were low levels of intraplatelet ADP and 5-hydroxytryptamine and high ATP/ADP ratios. Although these abnormalities occurred with both types of tumor, they were more frequent and marked for melanomas and breast carcinomas spread to regional lymph nodes. Our data indicate that the presence of exhausted platelets is an early finding in patients with malignant tumors.

Adenocarcinoma↗
PubMed1988

Hermansky-Pudlak syndrome: an immunologic assessment of 15 cases.

PURPOSE: The Hermansky-Pudlak syndrome is an autosomal recessive disorder consisting of the triad of oculocutaneous albinism, a storage pool platelet defect, and multisystem tissue deposition of ceroid pigment. Although the underlying metabolic defect has not been identified, secondary or associated effects on the immune system are suggested by reports of an association with disorders such as pulmonary fibrosis, granulomatous colitis, lupus, and frequent bacterial infections. We evaluated a large group of patients with the Hermansky-Pudlak syndrome to assess immune competence in this condition. PATIENTS AND METHODS: Fifteen patients with this syndrome were studied. Control subjects included healthy volunteers in the same age range as the patients. Peripheral blood lymphocytes and neutrophils were isolated according to previously described methods. Immunofluorescent staining, flow cytometry, and cytotoxicity assays were performed. Determination of lymphocyte transformation, mixed lymphocyte response, and neutrophil function was made. RESULTS: Immunoglobulin levels, complement, lymphocyte subsets, natural killer and lymphokine-activated cytotoxicity, mixed lymphocyte responses, and lectin-induced transformation were normal in all patients. In addition, there was no evidence for a lymphocyte proliferative response to a preparation of urinary ceroid pigment isolated from these patients. Neutrophil function, including luminol-dependent chemiluminescence, chemotaxis, and aggregation was not significantly different from control values. CONCLUSION: The results suggest that there is no generalized defect of peripheral blood lymphocyte or neutrophil function in the Hermansky-Pudlak syndrome. We propose that future studies should examine the possibility that associated disorders arise from a defect within the monocyte-macrophage system, perhaps secondary to ceroid accumulation.

Adolescent↗
PubMed1987

Inherited abnormalities of the platelet membrane and secretory granules.

Platelet stimulus-activation-contraction-secretion coupling is linked to fundamental modifications in the biochemistry and ultrastructure of the platelet surface and the membranes enclosing storage organelles. It is not surprising, therefore, that membrane defects are common in platelets from patients with inherited hemorrhagic disorders caused by platelet dysfunction. In fact, it might be stated that all inherited disorders of platelet function are related directly or indirectly to abnormalities of membranes. The current review discusses the state of knowledge on inherited platelet membrane defects of the cell surface and storage organelles.

Bernard-Soulier Syndrome↗
PubMed1992

Potentiation of uremic bleeding by hereditary storage pool disease.

This study demonstrates that specific bleeding tests can separate the thrombocytopathy of uremia alone from the bleeding disorders caused by uremia superimposed on preexisting platelet dysfunction. The case history of a uremic patient with exaggerated bleeding tendencies is presented. The findings in this patient are compared with the clinical characteristics and platelet function studies of nine other patients with chronic renal failure. The index and other uremic patients were similar except for the clinical bleeding and results of platelet function studies. The patient's nonocclusive bleeding time and measured blood loss during bleeding time tests were increased compared with the other uremic controls. In addition, her platelet aggregation in response to collagen was lower than that of the other uremic subjects. Repeat studies following renal transplantation were consistent with hereditary storage pool disease. An underlying platelet disorder may potentiate the hemostatic defects of uremia. The diagnosis should be suspected in patients with frequent and severe bleeding manifestations. Renal transplantation led to control of clinical bleeding.

Adult↗
PubMed1997

Megakaryocytes and platelets in alpha-granule disorders.

This chapter summarizes research data contributing to current understanding of disorders affecting alpha-granules of megakaryocytes and platelets. Diagnostic features of the gray platelet syndrome are well defined. Combined evidence suggests a defect, specific to the megakaryocyte cell lineage, causing a cytoskeletal abnormality and defective targeting of endogenously synthesized proteins to the alpha-granule. The abnormalities linked by signal transduction pathways. von Willebrand disease and afibrinogenaemia are disorders which highlight the functional importance of platelet storage pools of von Willebrand factor and fibrinogen, essential ligands in the process of adhesion and aggregation. The abnormality in the factor V Quebec disorder leads to a degradation of most proteins contained within the alpha-granule. The familial platelet disorder Paris-Trousseau thrombocytopenia is the only alpha-granule disorder associated with a cytogenetic abnormality, and it presents a useful model for exploring the genetic influence on regulation of thrombopoiesis. Study of these syndromes has elucidated aspects of the physiology of normal megakaryocyte maturation and platelet formation, including storage organelle biosynthesis.

Afibrinogenemia↗
PubMed2000

In vivo biotinylation studies: specificity of labelling of reticulated platelets by thiazole orange and mepacrine.

Animal in vivo biotinylation studies have demonstrated that thiazole orange (TO) labels the youngest cells in the circulation. TO has since been widely used for the measurement of reticulated platelets. As recent findings suggest that at high concentrations TO also labels platelet dense granules non-specifically, the value of previous work is unclear. Mepacrine also labels platelet dense granules and can detect storage pool defects. In this study, a mouse in vivo biotinylation model was used to determine the specificity of TO and mepacrine staining on platelets recently released into the circulation. The mean life span of biotin/TO (low), biotin/TO (high) and mepacrine/TO dual-positive platelets was 1.4 d (SD 0.5), 2.2 d (SD 0.2) and 2.3 d (SD 0.3) respectively (n = 6) compared with a life span for biotin-positive platelets of 4.9 d (SD 1.6). TO (low), TO (high) and mepacrine labelled 8.0% (SD 3.1), 43.9% (SD 8.3) and 40.0% (SD 9.9) of the total platelet population respectively (results of 30 samples from six mice), which decreased to 6.8% (SD 3. 9), 26.6% (SD 6.9) and 25.7% (SD 10.6) after thrombin degranulation. The shorter life span and lack of thrombin sensitivity of TO (low)-positive platelets, suggests that TO (low) measures reticulated platelets specifically. The comparative life spans and thrombin sensitivity of TO (high) and mepacrine-positive platelets suggest that TO (high) labels platelet dense granules. These data also suggest that dense granules are lost during platelet ageing.

Animals↗
PubMed2002

Cell-specific abnormal prenylation of Rab proteins in platelets and melanocytes of the gunmetal mouse.

The mutant gunmetal mouse exhibits reduced rates of platelet synthesis, abnormalities of platelet alpha and dense granules and hypopigmentation. Several of these features resemble those of human alpha/delta platelet storage pool disease, grey platelet syndrome and Hermansky-Pudlak syndrome. Gunmetal mice have reduced levels of Rab geranylgeranyltransferase (RabGGTase), which adds lipophilic prenyl groups to the carboxyl terminus of Rab proteins. The degree of prenylation and the subcellular distribution of several Rab proteins were evaluated in mutant platelets, melanocytes and other tissues. Significant deficits in prenylation and membrane binding of most Rabs were observed in platelets and melanocytes. In contrast, minimal alterations in Rab prenylation were apparent in several other gunmetal tissues despite the fact that RabGGTase activity was equally diminished in these tissues. The mutant tissue-specific effects are probably due to increased concentrations of Rab proteins in platelets and melanocytes. These experiments show that Rab proteins are differentially sensitive to levels of RabGGTase activity and that normal platelet synthesis, platelet organelle function and normal pigmentation are highly sensitive to the degree of prenylation and membrane association of Rab proteins. Further, the tissue-specific effects of the gunmetal mutation suggest that RabGGTase is a potential target for therapy of thrombocytosis.

Alkyl and Aryl Transferases↗
PubMed2000

Monthly haemoptysis in a woman with platelet storage pool disease.

A 26-year-old female with known platelet storage pool disease presented with a short history of recurrent haemoptysis. Initial investigations were unhelpful until the cyclical nature of the symptoms became apparent prompting the unusual diagnosis of pulmonary endometriosis to be made. This was subsequently confirmed on premenstrual CT scanning. The introduction of a specific hormonal therapy and multidisciplinary input was ultimately successful.

Adult↗
PubMed2000

Storage pool defect in pooled buffy coat platelet concentrates within the shelf-life period.

Platelet concentrate (PC) transfusions are useful for maintaining haemostasis in a variety of clinical situations. The function of transfused platelets is of critical importance, and changes on storage of buffy coat-prepared PC may influence their haemostatic potential. Total platelet adenine nucleotide content and platelet aggregation responses were studied, serially, in pooled buffy coat-derived PCs (n = 7), stored under UK recommended blood bank conditions, over the stipulated shelf-life of 5 days. Mean platelet volume (MPV), platelet counts and platelet distribution width (PDW) were also quantified. Total platelet ADP content decreased from 4.45+/-0.78 to 3.71+/-0.69 nmol/108 platelets (P<0.01, day 1 versus day 5, mean +/- SEM) over the shelf-life period. This was associated with reduced aggregatory responses: responses (expressed as percentage of maximum height) to 5 and 10 microM ADP decreased from 10.8+/-2.8% to 1.0+/-1.0% (P<0.005, 5 microM, day 1 versus day 5) and from 18.0+/-5.4% to 4.7+/-2.2% (P<0.02, 10 microM, day 1 versus day 5) while the decreased responsiveness was more pronounced for 4 microg/ml of collagen: 49.0+/-13.3% to 7.2+/-7.1% (P<0.01, day 1 versus day 4) and 49.9 +/-13.3% to 2.1+/-1.9% (P<0.001, day 1 versus day 5). These data indicate an acquired storage pool defect that is maximal by day 4 or 5 and accompanied by decreased platelet function, characterized by significant decreases in platelet aggregation responses. Addition of freeze-thawed plasma (autologous day 1) to PCs on days 2, 3, 4 and 5 did not alter the responses to ADP and collagen.

Adenosine Diphosphate↗
PubMed2006

Liver transplantation for liver rupture due to light chain deposition disease: a case report.

Light chain deposition disease (LCDD) is a rare pathologic condition distinct from amyloidosis. Amyloidosis is most often characterized by overproduction of lambda light chains, while kappa chains are overproduced in LCDD. In contrast to amyloid deposits, those of LCDD do not stain with Congo red and have a granular ultrastructure. LCDD primarily affects the kidney; clinically significant liver dysfunction is less common and less severe than renal disease. We describe a case of kappa chain deposition disease in a patient with plasma cell dyscrasia and platelet pool storage defect, which produced massive hepatomegaly and rupture of the liver leading to orthotopic liver transplantation. The liver weighed 6800 g and showed severe atrophy due to massive deposition of light chains. In this case, the deposits were composed of unbranched fibrils, which measured 12 to 20 nm in width, did not possess a hollow core, and were arranged randomly rather than in structured arrays.

Adult↗
PubMedDate not supplied

[Thrombocytic alpha-delta-storage-pool-disease: shortening of bleeding time after infusion of 1-desamino-8-D-arginine vasopressin].

BACKGROUND: The synthetic vasopressin derivate desmopressin (1-desamino-8-D-arginine vasopressin) has been reported to shorten the bleeding time in patients with hemophilia A, von Willebrand's disease and several functional platelet disorders. In addition to substitution of platelets, vasopressin is therefore used to prevent bleeding complications. CASE: We report the case of a 14-year-old female patient with prolonged bleeding time due to the rare thrombocytic alpha-delta-storage-pool-disease. When normal donor platelet substitution alone was ineffective, bleeding time was normalised after infusion of desmopressin and elective wisdom-tooth extraction was performed without significant postoperative bleeding. DISCUSSION: Infusion of desmopressin appears to be effective in shortening bleeding time in thrombocytic storage-pool-disease. Its use could prevent bleeding complications after trauma and surgical interventions and may possibly help to spare the need for platelet concentrates.

Adolescent↗