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.

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.

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.

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 “Almost Bleeding to Death”: The Conundrum of Acquired Amegakaryocytic Thrombocytopenia

2014 ◽  
Vol 2014 ◽  
pp. 1-5 ◽  
Author(s):  
Gabrielle Elena Brown ◽  
Hani M. Babiker ◽  
Carlos L. Cantu ◽  
Andrew M. Yeager ◽  
Ravitharan Krishnadasan
Keyword(s):  
Antithymocyte Globulin ◽  
Case Reports ◽  
Immunosuppressive Treatment ◽  
Severe Bleeding ◽  
Severe Thrombocytopenia ◽  
Disease Entity ◽  
Excessive Bleeding ◽  
Substantial Risk ◽  
Acquired Amegakaryocytic Thrombocytopenia

Acquired amegakaryocytic thrombocytopenia (AAT) is a rare hematological disorder causing severe thrombocytopenia and bleeding. Previous in vitro studies postulated both cell-mediated suppression of megakaryocytopoiesis in early megakaryocytic progenitor cells and humoral-mediated suppression by anti-thrombopoietin antibodies as possible etiologies of AAT. Patients with AAT usually present with severe bleeding and thrombocytopenia that is unresponsive to steroids and intravenous immunoglobulin (IVIG). Although standard guidelines have not been established for management of AAT, a few case reports have indicated a response to immunosuppressive treatment. The prompt recognition of this disease entity is essential in view of the substantial risk of morbidity and mortality from excessive bleeding. We report a case of AAT successfully treated with equine antithymocyte globulin (ATG) and cyclosporine (CSP).

A retrospective 11-year analysis of obstetric patients with idiopathic thrombocytopenic purpura

Blood ◽  
2003 ◽  
Vol 102 (13) ◽  
pp. 4306-4311 ◽  
Author(s):  
Kathryn E. Webert ◽  
Richa Mittal ◽  
Christopher Sigouin ◽  
Nancy M. Heddle ◽  
John G. Kelton
Keyword(s):  
Platelet Count ◽  
Retrospective Study ◽  
Epidural Analgesia ◽  
Idiopathic Thrombocytopenic Purpura ◽  
Vaginal Bleeding ◽  
Severe Bleeding ◽  
Thrombocytopenic Purpura ◽  
Platelet Counts ◽  
Itp In Pregnancy ◽  
In Pregnancy

AbstractNumerous studies have examined the outcomes of infants born to mothers with idiopathic thrombocytopenic purpura (ITP). Fewer studies have discussed the morbidity of obstetric patients with ITP. We describe a retrospective study of 92 women with ITP during 119 pregnancies over an 11-year period. Most women had thrombocytopenia during pregnancy. At delivery, women in 98 pregnancies (89%) had platelet counts lower than 150 × 109/L; most had mild to moderate thrombocytopenia. For many, the pregnancy was uneventful; however, women had moderate to severe bleeding in 25 pregnancies (21.5%). Women in 37 pregnancies (31.1%) required treatment to increase platelet counts. During delivery, 44 women (37.3%) received epidural analgesia without complications, with most having a platelet count between 50 and 149 × 109/L. Most deliveries (82.4%) were vaginal. Bleeding was uncommon at delivery. Infant platelet counts at birth ranged from 12 to 436 × 109/L; 25.2% of infants had platelet counts lower than 150 × 109/L, and 9% had platelet counts lower than 50 × 109/L. Eighteen infants (14.6%) required treatment for hemostatic impairment. Two fetal deaths occurred. One was caused by hemorrhage. ITP in pregnancy carries a low risk, but mothers and infants may require therapy to raise their platelet counts. (Blood. 2003;102:4306-4311)

Interleukin-10 Gene Polymorphism Reflects the Severity of Chronic Idiopathic Thrombocytopenic Purpura.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 2111-2111
Author(s):  
Takayuki Saitoh ◽  
Tetsuhiro Kasamatsu ◽  
Madoka Inoue ◽  
W.H.S. Al-ma’Quol ◽  
Akihiko Yokohama ◽  
...  
Keyword(s):  
Platelet Count ◽  
Idiopathic Thrombocytopenic Purpura ◽  
Response Rate ◽  
Initial Diagnosis ◽  
Control Group ◽  
Severe Thrombocytopenia ◽  
Bleeding Tendency ◽  
Thrombocytopenic Purpura ◽  
Chronic Itp ◽  
Significant Difference

Abstract Introduction: Recent several cytokine studies have shown Th1 polarization of the immune response in Idiopathic thrombocytopenic purpura (ITP) patients. IL–10 is most important factor regulating Th1 and Th2 cytokine synthesis and IL–10 polymorphism has been implicated in autoimmunity and tumorigenesis. We examined the single nucleotide polymorphisms (SNPs) in the promoter regions of the IL–10 genes in patients with ITP, and analyzed the relationship between IL–10 SNPs and clinical features. Patients and methods: Seventy-eight patients (male/female; 19/59, median age; 59.4) diagnosed as chronic ITP and 202 healthy controls were included. ITP with severe thrombocytopenia was defined as thrombocytopenia (platelet count < 10×109/L) at initial diagnosis of ITP. ALL patients gave written informed consent about the study. The platelet count was ranged from 1×109/L to 100×109/L at an initial diagnosis. In addition, 53 patients (67.9%) had bleeding tendency, and 20 patients (25.6%) had severe thrombocytopenia. Steroid treatment was given to 48 patients (61.5%), while splenectomy was applied to only 9 patients (11.5%). Genotyping in IL-10-1082G/A, -819C/T, −592A/C was determined by PCR based technique. Genotype and allele frequencies were compared between the study groups using χ2-test. The characteristics and laboratory features of the ITP patients with each IL-10 promoter polymorphism were compared using X2-tests and student t-tests. Probability values <0.05 were considered statistically significant. Results: The frequencies of the genotypes were as follows: GG (0%), GA (6%), and AA (94%) for −1082; CC (12%), CT (51%), and TT (37%) for −812; CC (12%), CA (51%), and AA (37%) for −592 loci. The frequencies of each haplotype were as follows: ATA/ATA haplotype in 31 patients (40%), ATA/ACC haplotype in 35 patients (45%), ACC/ACC haplotype in 7 patients (9%). No significant differences in the genotype or haplotype frequencies demonstrated between chronic ITP patients and control group. However, patients with −592AA genotypes showed severe thrombocytopenic state at initial diagnosis compared to those with −592CA/CC genotypes (41.4% vs. 16.3%, p=0.01). Furthermore, patients with ATA/ATA haplotype showed severe thrombocytopenic state (38.7% vs. 17%, p=0.03) compared to those without ATA/ATA haplotype. In patients treated with steroids, the overall response rate was 71% with complete response rate of 23.2% and partial response rate of 47.8%. No significant difference was observed in treatment response according to IL-10 polymorphism. Conclusion: In previous investigations, −592AA genotype or ATA/ATA haplotype have been reported to be associated with the lower levels of IL-10 expression. Our data suggest that the group with low IL-10 inducibility (i.e. −592AA genotype, ATA/ATA haplotype) may have more severe thrombocytopenia compared to those with high IL-10 inducibility. It is also reported that low IL-10 inducibility type enhances Th1-type polarization in ITP. Furthermore, Panitsas et al. revealed that higher Th1/Th2 ratio in ITP patients correlate with lower platelet count. Thus, these findings suggest that IL-10 polymorphism reflect the severity of chronic ITP.

scholarly journals Mononeuritis multiplex in acquired amegakaryocytic thrombocytopenia

2015 ◽  
Vol 06 (04) ◽  
pp. 588-590
Author(s):  
Firosh Khan ◽  
Abdulkhader Shehna ◽  
Lekha Mukundan
Keyword(s):  
Bone Marrow ◽  
Nervous System ◽  
Idiopathic Thrombocytopenic Purpura ◽  
Treatment Options ◽  
The Body ◽  
Bleeding Disorders ◽  
Bleeding Diathesis ◽  
Mononeuritis Multiplex ◽  
Thrombocytopenic Purpura ◽  
Acquired Amegakaryocytic Thrombocytopenia

ABSTRACTMononeuritis multiplex involves inflammation of two or more nerves, typically in unrelated parts of the body. It has been well described in bleeding disorders like idiopathic thrombocytopenic purpura (ITP) and Hemophilia. Acquired amegakaryocytic thrombocytopenia (AAT) is a bleeding diathesis characterized by thrombocytopenia but with reduced number of megakaryocytes in the bone marrow, as against ITP. Though AAT is a well described entity, peripheral nervous system manifestations have not been described so far. We report a young man who has presented with bleeding diathesis and mononeuritis multiplex due to AAT. The mechanism of development of mononeuritis multiplex and treatment options are discussed.

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.

The Influence of Polymorphisms of Interleukin-17F Genes on the Susceptibility and Clinical Significanse of Chronic Idiopathic Thrombocytopenic Purpura.

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 3428-3428
Author(s):  
Takayuki Saitoh ◽  
Tetsuhiro Kasamatsu ◽  
Akihiko Yokohama ◽  
Hiroshi Handa ◽  
Norifumi Tsukamoto ◽  
...  
Keyword(s):  
Platelet Count ◽  
Idiopathic Thrombocytopenic Purpura ◽  
Initial Diagnosis ◽  
Severe Thrombocytopenia ◽  
Single Nucleotide ◽  
Thrombocytopenic Purpura ◽  
Chronic Itp ◽  
Genotype Frequencies ◽  
Significant Difference ◽  
And Control

Abstract Introduction: Recent several cytokine studies have shown Th1 polarization of the immune response in idiopathic thrombocytopenic purpura (ITP) patients. Interleukin-17 F(IL-17F) is a relatively new cytokine that regulates the adaptive and innate immune systems. In vivo studies in murine disease indicate that the Th17 lineage plays a pathogenic role in autoimmune disease. IL-17 polymorphism has been implicated in autoimmunity, including ulcerative colitis and asthma. Polymorphisms were studied, including the coding-region sequence variant single nucleotide polymorphism rs763780 (7488T/C), which causes a His-to-Arg substitution at amino acid 161 (H161R). We examined the single nucleotide polymorphisms (SNPs) in the promoter regions of the IL-17 genes in patients with ITP, and analyzed the relationship between IL-17 SNPs and clinical features. Patients and methods: Seventy-eight patients (male/female; 19/59, median age; 59.4) diagnosed as chronic ITP and 202 healthy controls were included. ITP with severe thrombocytopenia was defined as thrombocytopenia (platelet count < 10X109/L) at initial diagnosis of ITP. ALL patients gave written informed consent about the study. The platelet count was ranged from 1X109/L to 100X109/L at an initial diagnosis. Genomic DNA was isolated from peripheral blood using the DNA Kit (QIAGEN, Hilden, Germany). Genotyping in IL-17F was determined by PCR based technique. Genotype and allele frequencies were compared between the study groups using χ2-test. The characteristics and laboratory features of the ITP patients with each IL-10 promoter polymorphism were compared using χ2- tests and student t-tests. Probability values <0.05 were considered statistically significant. Results: The frequencies of the genotypes were as follows: TT (72%), TC (12%), and CC (16%). No significant differences in the genotype frequencies demonstrated between chronic ITP patients and control group. However, patients with TT/TC genotypes showed severe thrombocytopenic state at initial diagnosis compared to those with CC genotypes (42.2% vs. 23.1%, p<0.05). No significant difference was observed in treatment response according to IL-17 polymorphism. Conclusion: No significant differences in the genotype frequencies demonstrated between chronic ITP patients and control. However, homozygosity of the H161R variant was inversely associated with severity of chronic ITP. Thus, these findings suggest that IL-17 polymorphism reflect the severity of chronic ITP.

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