scholarly journals Liver transplantation of a patient with extreme thrombocytopenia - A case report -

Author(s):  
Yena Oh ◽  
Seung Yeon Yoo ◽  
Gyu-Seong Choi ◽  
Gaab Soo Kim

Background: Patients with chronic liver disease (CLD) planned for liver transplantation (LT) often show severe thrombocytopenia, but there is a lack of evidence in deciding the threshold for prophylactic platelet transfusion.Case: A 47-year-old women with acute liver failure was referred for LT. Despite daily transfusion of platelets, platelet counts remained under 10,000/µl. During LT, 2 units of single donor platelets (SDP) were transfused. Although platelet counts remained extremely low (3,000–4,000/µl) no diffuse oozing was observed and the blood loss was 860 ml. Postoperatively, there was no sign of active bleeding or oozing, and the patient received only 1 unit SDP transfusion.Conclusions: CLD patients may have severe thrombocytopenia. However, primary hemostasis may not be significantly hindered due to the existence of rebalanced hemostasis. Prophylactic platelet transfusion in these patients should not be decided based on platelet counts only, but also take other coagulation tests and clinical signs into consideration.

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Hiroaki Nomoto ◽  
Naoki Morimoto ◽  
Kouichi Miura ◽  
Shunji Watanabe ◽  
Yoshinari Takaoka ◽  
...  

Abstract Background Chronic liver disease (CLD) is often complicated by severe thrombocytopenia (platelet count < 50,000/µL). Platelet transfusion has been a gold standard for increasing the platelet count to prevent hemorrhagic events in such patients. Lusutrombopag, a thrombopoietin receptor agonist, can increase the platelet count in such patients when invasive procedures are scheduled. Former studies on lusutrombopag included patients with a platelet count of > 50,000/µL at baseline: the proportions of patients who did not require platelet transfusion were 84–96%, which might be overestimated. Methods The efficacy and safety of lusutrombopag were retrospectively investigated in CLD patients with platelet count of < 50,000/µL, a criterion for platelet transfusion, in real-world settings. We examined the proportion of patients who did not require platelet transfusion in 31 CLD patients, which exceeded a minimum required sample size (21 patients) calculated by 80% power at a significance level of 5%. Lusutrombopag, 3 mg once daily, was administered 8–18 days before scheduled invasive procedures. Results Among 31 patients who received lusutrombopag, 23 patients (74.2%) patients showed a platelet count of ≥ 50,000/µL (Group A) and did not require platelet transfusion. The remaining 8 patients (25.8%) did not reached platelet ≥ 50,000/µL (Group B). The means of platelet increase were 38,000/µL and 12,000/µL in groups A and B, respectively. A low platelet count at baseline was a characteristic of patients in group B. Among 13 patients who repeatedly used lusutrombopag, lusutrombopag significantly increased the platelet count as the initial treatment. When all repeated uses of lusutrombopag were counted among these 13 patients, platelet transfusion was not required in 82.1% (23/28) of treatments. Although one patient showed portal thrombosis after lusutrombopag treatment, the thrombosis was disappeared by anticoagulant treatment for 35 days. The degree of platelet increase with lusutrombopag was larger than that in their previous platelet transfusion. Conclusions The proportion of patients who did not require platelet transfusion was 74.2%, which is smaller than that in former studies which included CLD patients with a platelet count of > 50,000/µL. However, lusutrombopag is effective and safe for CLD patients with a platelet count of < 50,000/µL.


Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 719-719
Author(s):  
Kathleen Pao Lynn Cheok ◽  
Rakchha Chhetri ◽  
Li Yan A Wee ◽  
Arabelle Salvi ◽  
Simon McRae ◽  
...  

Aim: Although 40-65% of myelodysplastic syndromes (MDS) patients are thrombocytopenic and require platelet transfusions, there is limited literature on the risk factors predictive of bleeding and the burden of immune mediated platelet refractoriness (PLT-R). Objectives: To evaluate the prevalence of thrombocytopenia, incidence of bleeding events, platelet transfusion dependency (PLT-TD) and immune-mediated platelet refractoriness (PLT-R) in MDS patients. Methods: A retrospective analysis of 754 MDS patients enrolled in the South Australian MDS (SA-MDS) registry was performed. Platelet counts &lt;100, &lt;50 and &lt;20 (x109/L) were used to define mild, moderate and severe thrombocytopenia respectively. The severity of bleeding events was classified according to the International Society of Thrombosis and Haemostasis (ISTH) classification. PLT-TD was defined as transfusion of at least one unit of platelets each month for four consecutive months. All other patients were classified as transfusion independent (PLT-TI). Immune mediated PLT-R was defined if a patient had HLA-class I or HPA antibodies, poor platelet increments and required HLA-matched platelets. Medication history with regards to anticoagulation and/or antiplatelet therapy was also collected. Results: At diagnosis, 332 (45%) patients had thrombocytopenia and 106 (14%) patients had moderate to severe thrombocytopenia. During the study period, 249 bleeding events were recorded in 162 (21%) patients with a cumulative incidence of 33% (Fig 1A). Of the 249 bleeding events, 85 (34%) were major and 164 (66%) were clinically relevant minor bleeding. Notably, 16, 90 and 5 bleeding events were intracranial, gastrointestinal, intraocular respectively. While 41% (96/235) bleeding events occurred in the setting of moderate to severe thrombocytopenia, 21% and 38% (Fig 1B) of bleeding events occurred at platelet counts of &gt;50-100 and &gt;100x109/L respectively. Twenty-eight percent (69/249) bleeding events were associated with concomitant anticoagulation and/or antiplatelet therapy and importantly, platelets counts were &gt;50x109/L and &gt;100 x109/L in 57 (83%) and 46 (67%) at the time of bleeding events, respectively. During the disease course, 393 (52%) patients required at least one unit of platelet transfusion. 106 (14%) patients were PLT-TD and had significantly poor overall survival (OS) compared to PLT-TI (26 vs 42 months, p&lt;0.0001). In total, 30/393 (7%) required HLA-matched platelet transfusions. 20/30 (66%) of PLT-R patients were female receiving disease modifying therapy (DMT). This was substantiated by cox regression analysis, demonstrating that females (HR=5.32, p=0.0006), older age (HR=0.97, p=0.028) and haemoglobin (Hb) at diagnosis (HR=1.03, p=0.009) were independent risk factors for PLT-R. Importantly, 20/76 (25%) female patients receiving platelets and DMT developed immune mediated PLT-R requiring HLA matched platelets. Conclusions: In our cohort of MDS patients, cumulative incidence of bleeding is 33% and 59% of the bleeding events occurred at platelet counts &gt;50X109/L. For all bleeding events that occurred while on anticoagulation and/or antiplatelet therapy, 83% events occurred with platelet counts &gt;50 x 109/L. Therefore, guidelines for anticoagulation and/or antiplatelet therapy are required for MDS patients. We also showed that development of PLT-TD is associated with poor OS. Importantly, 1 in 4 female MDS patients receiving platelets and DMT required HLA-matched platelets. Platelet transfusions practices should be optimised for these high-risk groups. Disclosures No relevant conflicts of interest to declare.


Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4293-4293
Author(s):  
Lakshminarayanan Nandagopal ◽  
Muthu Veeraputhiran ◽  
Tania Jain ◽  
Ayman Soubani ◽  
Charles A. Schiffer

Abstract Introduction Prophylactic platelet transfusions are often performed prior to bronchoscopy or broncho-alveolar lavage (BAL) to prevent bleeding in thrombocytopenic patients. There is a paucity of data to validate this approach, with a platelet transfusion threshold of <50,000/mm3 largely based on expert opinion. We conducted a retrospective study on the incidence of bleeding complications in thrombocytopenic patients undergoing bronchoscopy. Methods We identified 150 consecutive patients with platelet counts <100,000/mm3 who underwent bronchoscopy and/or BAL from January 2009 to May 2014 at our institution. Bronchoscopies performed in patients with frank hemoptysis and trans-bronchial lung biopsy procedures were excluded. Patient characteristics, underlying diagnosis, platelet count prior to bronchoscopy, administration of platelet transfusions and bronchoscopy details were recorded. Factors affecting bleeding risk including presence of renal dysfunction (defined as BUN >30 and/or Cr>2.0) and coagulation studies (PT, PTT, INR) were identified. The British Thoracic Society guidelines1 were used to categorize bleeding as a result of bronchoscopy. Data were analyzed using descriptive statistics. Results The median age was 59 years (range 27-90), with two-thirds of patients (63%) being male. One hundred and seventeen (78%) patients had underlying malignancy and 55 (37%) had thrombocytopenia related to malignancy. Fellows and residents under the supervision of a bronchoscopy certified attending performed all but 4 of the bronchoscopies. Infection (40%) was the primary indication for bronchoscopy with BAL performed in 127 (85%) patients. Fifty-eight of 89 (65%) patients with baseline platelet counts <50,000/mm3 received prophylactic transfusions compared to 8% of those with platelet counts >50,000/mm3. The platelet count did not rise to >50,000//mm3 in many transfused patients. Seventy patients (47%) had counts <50,000/mm3 and eighty patients (53%) had counts >50,000/mm3 at the time of bronchoscopy. 49% were receiving immunosuppressive medications, 45% had renal dysfunction and 8% had INR >1.5. Bloody lavage that resolved spontaneously without continuous suctioning (Grade 0) was observed in 9 (6%) patients. Bleeding that required continuous suctioning but then resolved spontaneously (Grade 1) was noted in 1 patient with a platelet count of 61,000/mm3. Of 10 total bleeding events, 7 occurred in patients who were intubated. Two additional patients with platelet counts of 30,000/mm3 and 53,000/mm3 had diffuse alveolar hemorrhage, which was present before bronchoscopy. “Old” blood and blood clots were observed in 6 patients. Discussion The low incidence of bleeding complications from bronchoscopy +/- BAL even in patients with platelet counts <30,000/mm3 (3 episodes in 31 patients, all grade 0) demonstrates that bronchoscopy can be safely done in severely thrombocytopenic patients. Adopting a lower threshold for prophylactic transfusions could save a considerable number of platelet units and translate into significant cost savings and decreased risk of transfusion-related complications. Table 1 Platelet count, transfusion history and bleeding complications during bronchoscopy Platelet count at the time of bronchoscopy Number (n) and percentage (%) of patients who underwent bronchoscopy Number of patients who received prior platelet transfusion Bleeding during bronchoscopy n % 0-15,000/mm3 9 6% (9/150) 5 Grade 0=1 pt 16-29 22 15% 16 Grade 0=2 pts 30-39 17 11% 9 Grade 0=1 pt 40-49 22 15% 9 Grade 0=3 pts 50-75 44 29% 14 Grade 1=1 pt 76-100 36 24% 10 Grade 0=2 pts Total 150 63 Grade 0=9 pts, Grade 1=1 pt. 1.Du Rand IA, Blaikley J, Booton R, et al. British Thoracic Society guideline for diagnostic flexible bronchoscopy in adults: accredited by NICE. Thorax. 2013:68 Suppl 1:i1-i44 Disclosures No relevant conflicts of interest to declare.


2020 ◽  
Vol 52 (6) ◽  
pp. 1950-1952 ◽  
Author(s):  
Ting Li ◽  
Xiaodong Sun ◽  
Feixiang Luo ◽  
Shifei Song ◽  
Ying Yu ◽  
...  

Author(s):  
Arockiaseeli Mabel Annarani. I ◽  
Amalorpavamari Lucas ◽  
Gnanadeepam Gnanadeepam ◽  
Shirley Ann. C ◽  
Christy Gunaseeli. S

Liver transplantation is a treatment option for patients with End-stage Liver disease for whom all possible modes of surgical and medical treatment have been exhausted. The indications for Liver transplantation can be either acute or chronic liver disease. The absolute contra indications are active alcohol or substance abuse, severe cardiopulmonary or other co morbid conditions that would preclude meaningful recovery after transplant. Patients are listed on the waiting list according to their blood type and Model of End- Stage Liver Disease (MELD) score. The surgical procedure entails the excision of both donor and recipient livers and transplantation of the donor liver into the recipient (Orthotopic Liver Transplantation). This article gives an overview about liver transplantation and its management. A case report is presented and the nursing care has been discussed in detail.


Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 4217-4217
Author(s):  
Barry E Rosenbloom ◽  
Sarah Kulke ◽  
John Taylor ◽  
Neal J. Weinreb

Abstract Abstract 4217 Background: Patients with Gaucher disease frequently experience a delay in diagnosis despite clinical signs and/or symptoms. This delay is thought to stem from the rarity of the disease and physicians' inexperience with the constellation of disease manifestations that often overlap those of more common hematological illnesses. It has been the authors' experience that, in the absence of palpable splenomegaly, physicians are even less likely to consider the possibility of Gaucher disease in patients who have other typical manifestations such as chronic bone pain or hematological cytopenias. Using data submitted to the International Collaborative Gaucher Group (ICGG) Gaucher Registry, the largest single repository of Gaucher disease clinical information in existence, we sought to determine how commonly such patients are likely to be encountered. Purpose: To determine the frequency of patients with Gaucher disease who have little to no splenomegaly at diagnosis despite having concurrent moderate or severe thrombocytopenia. Methods: Data were evaluated from all patients with Gaucher disease type 1 enrolled in the ICGG Gaucher Registry as of June 3, 2011 who had not been splenectomized before diagnosis and whose spleen volumes and platelet counts at the time of diagnosis were reported to the Registry. Splenomegaly was quantitated by MRI, CT or ultrasonic volumetric measurements and categorized as mild/none (≤5 multiples of normal [MN] based on a normal value of 0.2% of body weight), moderate (>5 to ≤15 MN) and severe (> 15 MN). Platelet counts were categorized according to thrombocytopenia categories of mild/none (≥120 ×103/mm3), moderate (>60 to ≤120 × 103/mm3) and severe (<60 × 103/mm3). Results: A total of 612 patients with platelet counts and spleen volumes were identified. Of these, 96/612 (15.7%) had mild or no splenomegaly at the time of Gaucher diagnosis. Of these 96 patients, 37 (38.5%) had moderate thrombocytopenia and 6 (6.3%) had severe thrombocytopenia. Conclusions: Type 1 Gaucher patients often present with moderate to severe thrombocytopenia but with either normal spleen volumes or with splenomegaly that is not likely to be clinically apparent. These patients may be at particular risk for diagnostic delay but no less susceptible to Gaucher disease morbidity than patients with a more typical presentation. Further description of this cohort with respect to genotype, demographics, and other disease manifestations will be presented. Disclosures: Rosenbloom: Genzyme Corporation ; Shire Pharmaceuticals: Research Funding, Speakers Bureau; Genzyme Corp: Research Funding, Speakers Bureau. Kulke:Genzyme, a Sanofi Company: Employment. Taylor:Genzyme, a Sanofi Company: Employment. Weinreb:Genzyme Corporation: Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding, Speakers Bureau; Shire HGT: Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding, Speakers Bureau; Actelion Corporation: Speakers Bureau.


Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 3428-3428
Author(s):  
Steven M. Marionneaux ◽  
Peter G. Maslak ◽  
Nenita Francisco ◽  
Virgil Chan ◽  
Justin Hanenberg ◽  
...  

Abstract Abstract 3428 Severe thrombocytopenia is not an uncommon finding in the setting of hematologic malignancies. The key to a successful outcome often lies in the ability to support the patient through periods of cytopenia by adopting an aggressive transfusion strategy for platelets. Therefore, an accurate assessment of the platelet count is of utmost importance particularly as it relates to various levels which “trigger” prophylactic platelet transfusion. However, the accuracy and precision of conventional platelet counting methods have been shown to be suspect in severely thrombocytopenic samples. We investigated the accuracy of several commercial hematology analyzers currently available in clinical practice which use optical, impedance and immunologic platelet determination methods and assessed whether biased results could have an impact on decisions regarding platelet transfusion. Four-hundred three (403) EDTA-anticoagulated samples collected from patients with hematologic malignancies and a platelet count of < 50 × 10e3/uL were selected from the standard workflow processed in the hematology laboratories at MSKCC. Samples were split and tested within 8 hours of collection using optical based methods on the Advia 2120i (Siemens, Tarrytown, NY) and CELL-DYN Sapphire (Abbott Diagnostics, Santa Clara, CA). During the study, an additional analyzer became available, the XE-2100 (Sysmex, Kobe, Japan) which enumerates platelets by impedance and optical technology. One hundred twenty seven (127) of the 403 samples were split and tested by the Sysmex analyzer. Platelet counts from each method were then compared using the CD61 immunoplatelet determination (Abbott, Santa Clara, CA) as the reference value. The CD61 immunoplatelet method was chosen as the reference standard because we found excellent correlation in linear regression between this method and phase microscopy (r =0.99, y = −0.96 + 0.88x; n = 37). Also, the International Counsel for Standardization in Haematology and the International Society for Laboratory Hematology recommend the counting of specifically labelled platelets (CD41, CD61) by flow cytometry as a reference method for the enumeration of platelets. Bland-Altman difference plots were used to visualize the agreement between the reference and test methods and the paired t-test evaluated the statistical significance of the difference between methods. We then compared the number of platelet transfusion indications (at various platelet thresholds) as determined by all methods with potential transfusion decisions made using the reference method. There was a statistically significant positive bias among the optical and impedance methods compared with CD61 enumeration. Using various platelet transfusion decision points, the number of patients who were at risk for under transfusion (platelet count above threshold when reference result is < threshold) ranged from 1–2 % at a threshold of 50k/uL to 30–40 % when the threshold was lowered to 10k/uL. These results highlight the limitations in the accuracy of optical and impedance methods, particularly in the setting of severe thrombocytopenia. Since prophylactic platelet transfusions are by and large based on standard “triggers,” an overestimation of the platelet count may lead to under-transfusion, especially in patients at the highest risk for hemorrhage. Clinicians should be aware of the limitations of automated platelet determinations and consider this when making medical decisions regarding supportive transfusions. Disclosures: Marionneaux: Abbott Diagnostics: Consultancy.


Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 4834-4834 ◽  
Author(s):  
Fabiana Mendes Conti ◽  
Ana Paula Hitomi Yokoyama ◽  
Marcia R Dezan ◽  
Thiago H Costa ◽  
Maria Giselda Aravechia ◽  
...  

Abstract Background Post-transfusion purpura (PTP) is a rare yet serious disease characterized by severe thrombocytopenia occurring after a blood transfusion. It is caused by alloimmunization against platelet antigens, anti-HPA-1a being the most frequent antibody. We report two cases of PTP and discuss the clinical presentation, diagnosis and management of this serious condition. Case Report Case 1) Female patient, 90 years old, Caucasian, had a fracture in the umerus and head trauma in 2012, followed by upper GI bleeding. Four days after receiving two units of packed RBCs, she presented with a low platelet count (14.000/mm3), petechiae, gum bleeding and oozing from venipuncture sites. The platelet count further dropped to 2.000/mm3the following day. HPA genotyping and HPA antibody identification by MAIPA were performed to investigate post-transfusion purpura, which showed: HPA1bb, 3aa, 5aa and 15ab and presence of antibodies anti-HPA-1a. IVig 400 mg/kG was infused in two consecutive days, with normal platelet counts observed after a week. Two of her three children had the same HPA1bb genotype and performed a matched platelet and RBC donation by apheresis. One matched RBC unit was transfused to correct symptomatic anemia caused by bystander hemolysis seven days after the first transfusion. No platelet transfusions were needed. Case 2) Female patient, 29 years old, afrodescendant, had a hemorrhagic shock after an emergency hysterectomy due to placenta percreta in 2012. She received 14 RBC units, 10 fresh frozen plasmas and 10 units of cryoprecipitate. Two days later, another surgery was necessary to remove a retained coagulum, and she received 10 units of random platelets. Five days later, the platelet count dropped to 5.000/mm3, followed by sudden anemia (Hb=9--> 4,5 g/dL), without clinical or laboratory signs of hemolysis or evidence of bleeding, with normal coagulation times. HPA genotyping and antibody identification by MAIPA were performed and showed an HPA1bb genotype and presence of an anti-HPA-1a antibody. No platelet transfusions were administered and IVig 500mg/Kg was prescribed for 2 days. Only washed RBC units were transfused thereafter. The platelet count rose four days after starting IVig to 134.000/mm3, yet a second course of IVig was necessary a week later to reach normal platelet counts. Discussion Both reports illustrate severe thrombocytopenia in patients who presented with sudden thrombocytopenia up to a week after blood transfusion. Platelet alloimmunization happens after exposure to HPA antigens by transfusion or pregnancy, and severe thrombocytopenia occurs as an anamnestic response after reexposure to platelet antigens in any blood product that contains contaminating platelet membranes. The thrombocytopenia may evolve as an autoimmune process, which may also cause hemolytic anemia due toa bystander phenomenom. Diagnosis is based on the identification of the antibody in the serum of a patient who lacks the corresponding antigen, anti-HPA-1a being the most common. IVig 0,5-1g/Kg for 2 days is the treatment of choice. The RBC units must be washed to avoid exposure to platelet membranes and recurrence of thrombocytopenia. In future transfusions, washed RBCs or HPA1bb blood products should be used. Disclosures: No relevant conflicts of interest to declare.


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