Regulation of Serum Thrombopoietin Levels by Platelets and Megakaryocytes in Patients with Aplastic Anaemia and Idiopathic Thrombocytopenic Purpura

1996 ◽  
Vol 76 (02) ◽  
pp. 156-160 ◽  
Author(s):  
Naoaki Ichikawa ◽  
Fumihiro Ishida ◽  
Shigetaka Shimodaira ◽  
Tomoyuki Tahara ◽  
Takashi Kato ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Platelet Count ◽  
Aplastic Anaemia ◽  
Idiopathic Thrombocytopenic Purpura ◽  
Immunosorbent Assay ◽  
Regulatory Mechanism ◽  
Inverse Correlation ◽  
Normal Subjects ◽  
Enzyme Linked Immunosorbent Assay ◽  
Thrombocytopenic Purpura

SummaryTo clarify the regulatory mechanism of thrombopoietin (TPO, c-Mpl ligand) in chronic thrombocytopenic conditions, we determined TPO levels in the sera of patients with aplastic anaemia (AA; n = 26) and idiopathic thrombocytopenic purpura (ITP; n = 32) by an enzyme-linked immunosorbent assay. Despite a similarity in platelet counts, serum TPO levels in the AA group were markedly higher than those in the ITP group: 20.41 ± 9.71 f mol/ml (mean ± SD) and 1.66 ± 0.55 f mol/ml, respectively, both of which were significantly elevated compared to normal subjects (n = 41; 1.22 ± 0.37). In both groups, serum TPO level showed an inverse correlation with the platelet count. We determined the megakaryocyte volume using bone marrow clot section and found that it was markedly small in the AA group; while in the ITP group it was augmented with a correlation to serum TPO level. Our findings suggest that TPO levels may be regulated not only by platelets but also megakaryocytes in AA and ITP.

scholarly journals Phospholipid Accumulation in Histiocytes of Splenic Pulp Associated with Thrombocytopenic Purpura

Blood ◽  
1961 ◽  
Vol 18 (1) ◽  
pp. 73-88 ◽  
Author(s):  
SIDNEY L. SALTZSTEIN
Keyword(s):  
Bone Marrow ◽  
Platelet Count ◽  
Idiopathic Thrombocytopenic Purpura ◽  
Lipid Accumulation ◽  
Thrombocytopenic Purpura ◽  
Normal Platelet Count ◽  
Normal Platelet

Abstract Accumulation of a lipid, histochemically a phospholipid, in the histiocytes of the splenic pulp was observed in seven patients with thrombocytopenic purpura. Six had classical idiopathic thrombocytopenic purpura with abundant megakaryocytes in the bone marrow. Splenectomy resulted in clinical and hematologic remissions in four of these six, continued thrombocytopenia in the fifth, and in the continued requirement of corticosteroid to maintain a reasonably normal platelet count in the sixth. The seventh patient, who died shortly after splenectomy, had marked hypoplasia of megakaryocytes. Similar lipid accumulation was not seen in more than 700 other spleens, removed for a variety of reasons, reviewed in this study. Platelet phagocytosis has been suggested as a source of the lipid.

A Case of Acquired Amegakaryocytic Thrombocytopenia with Anti-c-mpl Autoantibody: Comparison with Idiopathic Thrombocytopenic Purpura

2019 ◽  
Vol 142 (4) ◽  
pp. 239-243
Author(s):  
Bora Son ◽  
Hee sue Park ◽  
Hye Sook Han ◽  
Hee Kyung Kim ◽  
Seung Woo Baek ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Platelet Count ◽  
Rare Disease ◽  
Idiopathic Thrombocytopenic Purpura ◽  
Immunosuppressive Treatment ◽  
Severe Bleeding ◽  
Severe Thrombocytopenia ◽  
Thrombocytopenic Purpura ◽  
Acquired Amegakaryocytic Thrombocytopenia

Acquired amegakaryocytic thrombocytopenia (AAMT) is a rare disease that causes severe bleeding. The pathogenesis and treatment of AAMT have not yet been defined. We report the case of a 60-year-old woman diagnosed with AAMT, who presented with severe thrombocytopenia, gastroin­testinal bleeding, and significantly reduced bone marrow megakaryocytes. The patient was treated with methylprednisolone, cyclosporin, and intravenous immunoglobulin. After 2 weeks of treatment, her platelet count started to increase, and her bone marrow megakaryocyte count had normalized 3 months after diagnosis. At the time of diagnosis, the patient was seropositive for anti-c-mpl antibody but was seen to be seronegative once the platelet count recovered. In contrast, anti-c-mpl antibodies were not detected in the serum of 3 patients with idiopathic thrombocytopenic purpura. This case study suggests that anti-c-mpl antibody plays an important role in the development of AAMT, and that intensive immunosuppressive treatment is required for autoantibody clearance and recovery of megakaryocyte count.

Concentrations of Thrombopoietin in Bone Marrow in Normal Subjects and in Patients With Idiopathic Thrombocytopenic Purpura, Aplastic Anemia, and Essential Thrombocythemia Correlate With Its mRNA Expression of Bone Marrow Stromal Cells

Blood ◽  
1998 ◽  
Vol 92 (1) ◽  
pp. 46-52 ◽  
Author(s):  
Yasuo Hirayama ◽  
Sumio Sakamaki ◽  
Takuya Matsunaga ◽  
Takashi Kuga ◽  
Hiroyuki Kuroda ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Aplastic Anemia ◽  
Idiopathic Thrombocytopenic Purpura ◽  
Essential Thrombocythemia ◽  
Stromal Cells ◽  
Detection System ◽  
Normal Subjects ◽  
Normal Range ◽  
Thrombocytopenic Purpura ◽  
Polymerase Chain

The function of bone marrow (BM) stromal thrombopoietin (TPO) in megakaryopoiesis remains unknown. In the present study we attempted to clarify the pathophysiological implications of stromal TPO in normal subjects (NS) and in patients with idiopathic thrombocytopenic purpura (ITP), aplastic anemia (AA), and essential thrombocythemia (ET) by measuring TPO concentrations in BM and peripheral blood (PB) and by estimating the levels of stromal TPO mRNA with TaqMan fluorescence-based post–reverse transcription-polymerase chain reaction product detection system. The results showed that TPO concentrations in PB were significantly elevated in patients with ITP (34.9 ± 11.7 pg/mL) and AA (364.1 ± 153.5 pg/mL) but within normal range in patients with ET (each 20.0 and 22.1; NS, 22.1 ± 8.2 pg/mL). In all subjects, the TPO concentrations in BM correlated well with the PB levels, and the former were consistently higher than the latter. The concentrations of TPO in BM also correlated with the levels of TPO mRNA in stromal cells. Furthermore, expression levels of TPO mRNA clearly correlated with megakaryocyte counts in NS and patients with ITP, indicating that stromal TPO actually enhances megakaryopoiesis. Thus, our results in the present study indicate that TPO from BM stromal cells is considered to play an essential role for megakaryopoiesis under various patho-physiological conditions.

Presentation and Outcome of Idiopathic Thrombocytopenic Purpura in Children: A Single Institution Experience

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 4683-4683
Author(s):  
Kari Bradham ◽  
Felicia L. Wilson ◽  
Hamayun Imran
Keyword(s):  
Bone Marrow ◽  
Platelet Count ◽  
Idiopathic Thrombocytopenic Purpura ◽  
Conflicts Of Interest ◽  
Laboratory Data ◽  
Bone Marrow Examination ◽  
Published Data ◽  
Thrombocytopenic Purpura ◽  
Chronic Itp ◽  
Increased Risk

Abstract Abstract 4683 PURPOSE: To review the presenting features, response to treatment and outcome in children diagnosed with idiopathic thrombocytopenic purpura (ITP) at the University of South Alabama, Children’s and Women’s Hospital and Specialty Clinic. METHODS: Using ICD code 287.3, data were collected from the specialty clinic’s medical records and hospital database for children diagnosed with ITP between January 2005 and September 2010. Recurrent and chronic ITP were defined as thrombocytopenia recurring within or more than 6 months of diagnosis, respectively. Univariate and multivariate logistic regression analyses were performed to evaluate variables associated with chronic ITP. RESULTS: Eight four patients were identified (M,F 1:1) with an average age of 70 months at diagnosis. Mean platelet count at presentation was 14k. Oral or nasal mucosal bleeding occurred in 19(23%) patients but none experienced a serious hemorrhage. Thirty three (39%) patients had an associated illness prior to diagnosis of ITP. Treatment consisted of intravenous immunoglobulin (IVIG) in 38(45%), WinRho in 11(13%), IVIG or WinRho followed by the other in 20(24%), data not available 8(10%) and no therapy in 7 patients (8%). Average platelet count at discharge and within 2 weeks after IVIG and WinRho was 57k, 337k and 57k, 375 respectively. Forty three (51%) were acute, 17(20%) became recurrent, and 24(29%) became chronic ITP. Bone marrow examination was performed in 26(30%) patients upon subsequent relapse but the diagnosis remained unaltered in all cases. Rituximab therapy was provided to five and splenectomy was performed in 7 patients. Four patients failed both modalities, all of whom currently are IVIG dependant. Age <5year (OR 0.12, 95%CI 0.22, 0.67, p=0.01) was protective against development of chronic ITP while platelet count >20k at presentation (OR 6.50, 95%CI 1.35, 31.30, p=0.02) and race other than white (OR 36.63, 95%CI 4.61, 291.09, p=0.001) were found to be significantly associated with the development of chronic ITP. Gender, mean platelet volume, total white cell count, and absolute lymphocyte count had no significant association. CONCLUSION: Our study supports the published data that patients with an initial platelet count >20k, older age and non-white race have an increased risk of progression to chronic ITP. Other published variables had no significant association in our analyses. Response to IVIG and WinRho was no different in our patients while rituximab or splenectomy did not lead to a complete resolution in refractory cases. Since bone marrow examination did not alter the diagnosis in any patient, we suggest that routine performance of this procedure may be omitted when a diagnosis of ITP is consistent with clinical history, physical examination and laboratory data. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Study of Bone Marrow Response to Platelet Transfusion in Severe Traumatic Brain Injury Patients

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 4999-4999
Author(s):  
Arulselvi Subramanian ◽  
Venencia Albert ◽  
Deepak Agrawal ◽  
Hara Prasad Pati
Keyword(s):  
Bone Marrow ◽  
Platelet Count ◽  
Brain Injury ◽  
Platelet Transfusion ◽  
Conflicts Of Interest ◽  
Inverse Correlation ◽  
Enzyme Linked Immunosorbent Assay ◽  
Study Cohort ◽  
Severe Tbi ◽  
Clinical Conditions

Abstract Introduction: SevereTraumatic Brain Injury (TBI) patients are predisposed to secondary brain insults due to thrombocytopenia and coagulopathy, warranting platelet transfusion. Thrombopoietin (Tpo) has been shown to be a physiological regulator of thrombopoiesis. An inverse correlation between Tpo and the circulating platelet has been previously reported for several clinical conditions. Response pattern of Tpo to circulating platelets in TBI patients has not been studied yet. We hypothesised that in severe TBI patients thrombopoiesis response to TPO will be impaired Methodology: Plasma Tpo levels were measured by enzyme-linked immunosorbent assay was measured 3 times i.e. Pre-platelet transfusion of platelets,24h and 48h post-transfusion in 100 severe (GSC≤8)TBI patients. Platelet transfusion response was evaluated and defined as a ≥20% increase in platelet count from baseline and patients were categorized as responders and non-responders to platelet transfusion. Results: Platelet count was low at admission (89.4±31.1*103 per cumm). Less than 2 units of random donor platelets(RDP) was given to 14% patients,67% patients required2-4 units of RDP and 19% required >4 units of RDP. TPO levels decreased 24h post transfusion compared to pre transfusion (Mean±SD)(127.8±90.2 vs. 142.2±82.6 pg/ml; P 0.07), but elevated at 48h (209.1±74.8 pg/ml; p Pre vs. 48h 0.05). On Average the platelet count improved after transfusion at 24h to 112.7±74.6pg/ml (p Pre vs. 24h 0.003) and continued to rise at 48 h (123.7±82.1 pg/ml; p Pre vs. 48h 0.02). Platelet count and TPO levels were found to be independent (rho -0.12, p 0.38). 62% showed a ≥20% increase in platelet count from baseline (Responders), 38% were non-responders. As significant increase in 24 h post transfusion Tpo levels were seen in responders [median (IQR)] [173.6(0-202.4) pg/ml vs. 164.3(137.1-203.7) pg/ml; p 0.04] compared to pre transfusion levels. Tpo levels in Non-responders did not vary post platelet transfusion [156(0-198.11) pg/ml vs. 158.88(0-198.11) pg/ml; p 0.97]. 28% of Non-responders had low (<81.25 pg/ml) and 2.8% had high (>237.7 pg/ml) pre transfusion Tpo levels. At 24 h post transfusion 26.6% had low and zero had High Tpo levels. Conclusion: In the present study platelet transfusion response inversely correlates with Tpo levels, as 24h post platelet transfusion Tpo levels were transiently low but insignificant and elevated at 48h post transfusion. In non-responders, the post transfusion TPO levels were not sufficient to mitigate the low platelet counts. Hence, the possibility of transient bone marrow depression in severe TBI patients is suggested, which requires further analysis in a larger study cohort. Disclosures No relevant conflicts of interest to declare.

Use of high-altitude climate in therapy of childrenwith idiopathic thrombocytopenic purpura in Kyrgyz Republic

2018 ◽  
Author(s):  
С.М. Маматов ◽  
А.К. Эсенгелди ◽  
А.А. Махмануров
Keyword(s):  
Platelet Count ◽  
High Altitude ◽  
Idiopathic Thrombocytopenic Purpura ◽  
Disease Duration ◽  
Hematological Parameters ◽  
Kyrgyz Republic ◽  
Duration Of Treatment ◽  
Thrombocytopenic Purpura ◽  
Hypoxia Exposure ◽  
The Subject

Введение. Вопрос о дальнейшем совершенствовании базисной терапии идиопатической тромбоцитопенической пурпуры (ИТП) по-прежнему широко обсуждается специалистами и является предметом оживленных дискуссий. Цель исследования: изучить динамику геморрагического синдрома и количества тромбоцитов у детей с ИТП в процессе высокогорной климатотерапии и оценить эффективность воздействия высокогорной гипоксии на течение болезни. Материалы и методы. В исследование включено 24 ребенка с хронической ИТП в возрасте от 5 до 14 лет (средний возраст — 10,25 ± 1,43 года) с длительностью заболевания от 4 до 8 лет. Для лечения детей поднимали на высокогорную базу Туя-Ашу (перевал Туя-Ашу, 3200 м над уровнем моря). Продолжительность лечения в высокогорье составляла 40 дней. Результаты. К концу срока пребывания в горах значительно уменьшались проявления геморрагического синдрома, полностью купировался анемический синдром, количество тромбоцитов достоверно увеличивалось с минимума 22,1 × 109/л до максимума 108,4 × 109/л. Заключение. Из 22 детей, получивших высокогорную климатотерапию, у 2 детей достигнута полная и у 15 — частичная ремиссия. У 4 детей улучшение клинико-гематологических показателей носило временный характер, и отсутствие эффекта зарегистрировано у 1 ребенка. Ремиссия достигнута у 77% больных детей. Introduction. Further improvement of the basic therapy of idiopathic thrombocytopenic purpura (ITP) is still widely discussedby specialists and is the subject of lively discussions. Aim: to study the dynamics of hemorrhagic syndrome and platelet count in children with ITP in the process of high-altitude climatotherapy and assess the eff ectiveness of high-altitude hypoxia exposure on the course of the disease. Materials and methods. The study included 24 children with chronic ITP aged 5 to 14 years (mean age — 10.25 ± 1.43 years) with disease duration from 4 to 8 years. For treatment children were raised to Tuya-Ashu high-altitude base (mountain pass Tuya Ashu, 3200 m above sea level). The duration of treatment in highlands was 40 days. Results. By the end of the treatment, the manifestations of hemorrhagic syndrome decreased signifi cantly, the anemic syndrome completely stopped, the platelet count increased significantly from minimum of 22.1 × 109/L to maximum of 108.4 × 109/L. Conclusion. High-altitude climatotherapy received 22 children, 2 children had complete remission and 15 — part remission. In 4 children the improvement of clinical and hematological parameters was temporal, and the absence of eff ect was registered in 1 child. Remission was achieved in 77% of ill children.

Clinical Study of TJ-137 Tsumura Kami-kihi-to in Patients with Idiopathic Thrombocytopenic Purpura (ITP)

1996 ◽  
Vol 2 (3) ◽  
pp. 213-218
Author(s):  
Nobuo Sakuragawa ◽  
Kojiro Yasunaga ◽  
Takeo Nomura ◽  
Junichi Akatsuka ◽  
Atsushi Kuramoto ◽  
...  
Keyword(s):  
Platelet Count ◽  
Clinical Study ◽  
Idiopathic Thrombocytopenic Purpura ◽  
Adverse Reactions ◽  
Clinical Effect ◽  
Thrombocytopenic Purpura ◽  
New Drug ◽  
Chronic Itp ◽  
Low Incidence

TJ-137 administered to patients with chronic ITP increased the platelet count "slightly" or more in 31.7% of the patients and showed a clinical effect in 40.9% with a rating of "modestly effective" or better. With a low incidence of adverse reactions, TJ-137 is ex pected to be a new drug for the treatment of ITP.

scholarly journals Autoimmune Hepatitis with Concomitant Idiopathic Thrombocytopenic Purpura Diagnosed by Transjugular Liver Biopsy

2018 ◽  
Vol 2018 ◽  
pp. 1-6
Author(s):  
Hiromi Fukuda ◽  
Kazuhide Takata ◽  
Takanori Kitaguchi ◽  
Ryo Yamauchi ◽  
Hideo Kunimoto ◽  
...  
Keyword(s):  
Platelet Count ◽  
Liver Biopsy ◽  
Autoimmune Hepatitis ◽  
Histological Examination ◽  
Idiopathic Thrombocytopenic Purpura ◽  
Liver Dysfunction ◽  
Severe Thrombocytopenia ◽  
Percutaneous Liver Biopsy ◽  
Thrombocytopenic Purpura ◽  
Transjugular Liver Biopsy

Patients with autoimmune hepatitis (AIH) may sometimes have concomitant idiopathic thrombocytopenic purpura (ITP). Severe thrombocytopenia in ITP interferes with percutaneous liver biopsy for pathological diagnosis of AIH. Here, we report a case of AIH with ITP in a 63-year-old woman. The patient presented to our hospital with liver dysfunction and thrombocytopenia. For histological examination, transjugular liver biopsy (TJLB) was performed, leading to a diagnosis of AIH. Corticosteroids treatment led to an improvement in her liver enzyme levels and platelet count. In conclusion, patients with AIH may sometimes have concomitant ITP. TJLB was effective for making the diagnosis of AIH with severe thrombocytopenia due to ITP.

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