Acquired Amegakaryocytic Thrombocytopenia Misdiagnosed as Immune Thrombocytopenia in a Patient with Seronegative Arthritis: A Case Report.

Acquired amegakaryocytic thrombocytopenia (AATP) is an uncommon cause of severe thrombocytopenia with preserved cells of other lineages, which can present with severe bleeding episodes. We report a case of a 45-year-old male with seronegative arthritis who was diagnosed with idiopathic thrombocytopenic purpura (ITP) and was being treated with steroids for ITP. Despite aggressive treatment, the patient had persistently low levels of platelets. In view of persistent thrombocytopenia, bone marrow biopsy was done and was diagnosed as Acquired Amegakaryocytic Thrombocytopenia (AATP). Patient was successfully treated with cyclosporine. Correct identification of AATP is essential because it can lead to life threatening bleeding manifestations and advance into Aplastic anemia or MDS. How to cite this article: N AM, Rajanna AH, Kamath N. Acquired Amegakaryocytic Thrombocytopenia Misdiagnosed as Immune Thrombocytopenia in a Patient with Seronegative Arthritis: A Case Report. J Assoc Physicians India 2023;71(11):100-102.

El volumen medioplaquetario: su importancia en la práctica clínica / Mean platelet volume: its importance in clinical practice

Las plaquetas son fragmentos citoplasmáticos anucleados derivados de los megacariocitos que presentan función central, tanto en procesos fisiológicos como la hemostasia, así como también en procesos patológicos como la inflamación, la ateroesclerosis. El tamaño de las plaquetas, medido como volumen medio de plaquetas, es un marcador de reactividad plaquetaria, que proporciona información importante sobre el curso y pronóstico de una variedad de afecciones inflamatorias. El estudio de este marcador se ha utilizado como pronóstico en enfermedades cardiovasculares, preeclampsia y en la púrpura trombocitopénica. El objetivo de este trabajo es revisar los conceptos actuales basados en la evidencia científica relacionados con el volumen plaquetario medio e insistir en su evaluación y significado en la práctica clínica cotidiana(AU)

Platelets are enucleated cytoplasmic fragments derived from megakaryocytes, which have a central function, both in physiological processes such as hemostasis, as well as in pathological processes such as inflammation, atherosclerosis. Platelet size, measured as mean volume of platelets, is a marker of platelet reactivity, providing important information on the course and prognosis of a variety of inflammatory conditions. The study of this marker has been used as a prognostic in cardiovascular diseases, preeclampsia and in thrombocytopenic purpura. The objective of this paper is to review the current concepts based on scientific evidence related to the mean platelet volume and to insist on its evaluation and meaning in daily clinical practice(AU)

Acquired amegakaryocytic thrombocytopenia after durvalumab administration.

Immune checkpoint inhibitors (ICIs), despite their ability to potentiate antitumor T-cell responses, may cause various immune-related adverse events. Most cases of thrombocytopenia induced by ICIs have revealed a pathophysiologic mechanism of immune thrombocytopenia with increased platelet destruction and preserved megakaryocytes. Acquired amegakaryocytic thrombocytopenic purpura (AATP) is an unusual disorder characterized by thrombocytopenia with markedly diminished bone marrow megakaryocytes in the presence of otherwise normal hematopoiesis. AATP caused by ICIs has not been reported on. Herein, we present the case of a 79-year-old man diagnosed with squamous cell carcinoma of the lung who developed AATP after two courses of durvalumab, a drug targeting programmed death-ligand 1. Two weeks after the second cycle, his platelet count decreased to 2.1 × 104/µL. After the patient underwent platelet transfusion, his platelet count increased to 8.1 × 104/µL the next day but subsequently decreased repeatedly even after the ICI was discontinued. Six weeks after the second cycle, he developed interstitial pneumonia and was administered prednisolone (50 mg/day). However, thrombocytopenia did not improve. Bone marrow biopsy showed scarce megakaryocytes (< 1 megakaryocyte/10 high-power fields) with preservation of myeloid and erythroid series. Myelodysplasia, myelofibrosis, or metastatic lesions were not observed. Cytogenetic analysis showed a normal male karyotype of 46XY. Hence, the patient received eltrombopag, a thrombopoietin receptor agonist, and his platelet count subsequently improved. After recovery, bone marrow aspiration revealed a normal number of megakaryocytes. AATP is rarely the type of thrombocytopenia induced by ICIs and may be successfully treated with thrombopoietin receptor agonists.

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

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, gastroin-testinal 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.

Thrombotic Microangiopathy and the Kidney.

Thrombotic microangiopathy can manifest in a diverse range of diseases and is characterized by thrombocytopenia, microangiopathic hemolytic anemia, and organ injury, including AKI. It can be associated with significant morbidity and mortality, but a systematic approach to investigation and prompt initiation of supportive management and, in some cases, effective specific treatment can result in good outcomes. This review considers the classification, pathology, epidemiology, characteristics, and pathogenesis of the thrombotic microangiopathies, and outlines a pragmatic approach to diagnosis and management.

Acquired amegakaryocytic thrombocytopenia previously diagnosed as idiopathic thrombocytopenic purpura in a patient with hepatitis C virus infection.

We report the first case of a patient with hepatitis C virus (HCV) infection and idiopathic thrombocytopenic purpura (ITP), who later developed acquired amegakaryocytic thrombocytopenia (AAMT), with autoantibodies to the thrombopoietin (TPO) receptor (c-Mpl). A 64-year-old woman, with chronic hepatitis C, developed severe thrombocytopenia and was diagnosed with ITP. She died of liver failure. Autopsy revealed cirrhosis and liver carcinoma. In the bone marrow, a marked reduction in the number of megakaryocytes was observed, while other cell lineages were preserved. Therefore, she was diagnosed with AAMT. Additionally, autoantibodies to c-Mpl were detected in her serum. Autoantibodies to c-Mpl are one of the causes of AAMT, acting through inhibition of TPO function, megakaryocytic maturation, and platelet formation. HCV infection induces several autoantibodies. HCV infection might also induce autoantibodies to c-Mpl, resulting in the development of AAMT. This mechanism may be one of the causes of thrombocytopenia in patients with HCV infection.

[Acquired amegacaryocytic thrombocytopenic purpura hiding acute myeloid leukemia]. / Un purpura thrombopénique amégacaryocytaire acquis qui cache une leucémie aigue myéloblastique.

Acquired amegakaryocytic thrombocytopenic purpura is a very rare condition characterized by severe thrombocytopenia linked to the reduction or disappearance of megakaryocytes in the bone marrow. It may be primary idiopathic or secondary to many pathological conditions including hematologic disorders. We report the case of a 24-year-old patient admitted for haemorrhagic syndrome caused by immunological thrombocytopenic purpura. The diagnosis was acquired amegakaryocytosis after the failure of corticotherapy and the performance of myelography. The patient was treated with ciclosporin with rapid progression to acute myeloblastic leukemia. The progression of acquired amegakaryocytosis to acute leukemia is reported but it is generally not so rapid and above all it is preceded by myelodysplastic syndrome or medullary aplasia. This study highlights the importance of a close follow-up of these pathologies with a benign-like appearance.

Robotic surgery for atrial septal defect closure in a case of Kabuki syndrome.

Kabuki syndrome is a rare congenital malformation syndrome characterized by mental retardation, skeletal deformities, auditory dysfunction, cardiac defects, and distinctive facial appearance. Although complex cardiovascular malformations present in early childhood, rarely, atrioventricular septal defects may also present in young adults. Presently described is case of a 22-year-old female with KS who presented with ostium secundum atrial septal defect with deficient rim and idiopathic thrombocytopenic purpura. In this case, minimally invasive robotic surgery was preferred for closure of atrial septal defect.

Acquired Amegakaryocytic Thrombocytopenic Purpura Progressing into Aplastic Anemia.

Acquired amegakaryocytic thrombocytopenic purpura (AATP) is a rare hematological disorder characterized by severe thrombocytopenia and a complete or near-to complete absence of megakaryocytes in the bone marrow, while granulopoiesis, as well as erythropoiesis are usually preserved. Although autoimmune mechanisms are believed to be causative, the exact underlying pathogenesis is not known. To date, only few cases have been reported and management of this disease remains controversial with immunosuppression being the treatment modality of choice in the majority of patients. In this article, we report a case of newly acquired AATP without an associated autoimmune disease, refractory to corticoids, intravenous immunoglobulin (IVIG) and second-generation TPO (thrombopoietin) agonists, which have recently been approved for the treatment of thrombocytopenia. Finally, in accordance with other reports, disease progression into aplastic anemia has occurred.

Two cases of thrombocytopenic purpura at onset of Zika virus infection.

We report here two cases of thrombocytopenic purpura at onset of Zika virus infection. A 26-year-old woman and a 21-year-old man had thrombocytopenia above 5×10(9) platelets/L. Hemorrhagic symptoms were mucosal and subcutaneous bleeding and gross hematuria and they reported episode of conjunctivitis. In both cases blood and bone marrow analysis suggested thrombocytopenic purpura, blood PCR tests for Dengue (DENV), Chikungunya (CHIKV) and Zika virus (ZIKV) were negative. In both cases urinary PCR for ZIKV was positive, Prednisolone yielded early remission. Only three similar cases have been reported so far. In the Caribbean, DENV is also epidemic and responsible for severe thrombocytopenia. Coinfections can occur. Our report underlines the need to include a ZIKV assay in the diagnostic work-up of thrombocytopenic purpura in epidemic areas.

A Seeming Paradox: Ischemic Stroke in the Context of Idiopathic Thrombocytopenic Purpura.

Nowadays, we have a relatively sophisticated standard approach to a patient with acute ischemic stroke, including the sequence of diagnostic methods and treatment modalities. In practice, however, we are occasionally confronted with a patient whose medical history or comorbidities force us to make a decision without the support of guidelines. One such situation is the occurrence of acute ischemic stroke in a patient with known idiopathic thrombocytopenic purpura, where a tendency to use thrombolysis, anticoagulants, or antiplatelet agents collides with the fear of life-threatening bleeding. In this review, we try to outline current understanding of the pathophysiology of "paradoxical" ischemic events in this illness characterized by thrombocytopenia and to summarize clinical experience from case reports dealing with this topic, which could help us to rely on more than individual opinion seen through a purely "neurological" or "hematological" prism.

Rifampcin-induced Thrombocytopaenia Purpura.

We report the case of a 28-year-old resident doctor with no past history of having taken rifampicin, who presented with thrombocytoapaenic purpura occurring after the initiation of anti-tuberculosis therapy (isoniazid, rifampicin, pyrazinamide and ethambutol) for tubercular lymphadenopathy.

Diagnóstico y tratamiento de la púrpura trombocitopénica inmunológica / Diagnosis and treatment of Immune thrombocytopenic purpura

Desde siempre los episodios de sangrado muco-cutáneos, al ser tan evidentes, causan mucha preocupación y zozobra, más aún si ocurren sin causa aparente. La púrpura trombocitopénica suele ser la responsable de estos desagradables sucesos. Esta revisión tiene por objetivo actualizar los conocimientos acerca de la fisiopatología, el diagnóstico y el tratamiento de la púrpura trombocitopénica inmunológica (PTI), una patología hematológica que afecta tanto a niños como a adultos y que se ve con relativa frecuencia en la actividad diaria de un hospital general...

Thrombocytopenic purpura is mostly responsible for episodes of muco-cutaneous bleeding. This review updates topics on the pathophysiology, diagnosis and treatment of immunologic thrombocytopenic purpura (IPT), an hematologic condition that afects both childern and adults, which is seen relatively frequent in daily practice in a general hospital...

Sulfamethoxazole-induced thrombocytopenia masquerading as posttransfusion purpura: a case report.

BACKGROUND: Drug-induced immune thrombocytopenia (DITP) is a rare clinical disorder characterized by accelerated platelet (PLT) clearance in the presence of drug-dependent antibodies. Distinguishing DITP from other immune-mediated disorders such as posttransfusion purpura (PTP) and autoimmune thrombocytopenia can represent a clinical challenge. CASE REPORT: A 68-year-old male with no prior transfusion history presented to the emergency department (ED) with dyspnea, epistaxis, and severe thrombocytopenia (<10 × 10(9)/L) 12 days after discharge from a hospital admission for a coronary artery bypass graft. Evaluation of the degree of thrombocytopenia and the temporal association between the peri- and postoperative receipt of multiple transfusions and the acute decrease in PLT count indicated PTP as a possible cause of the severe thrombocytopenia. Treatment with 1 g/kg intravenous immunoglobulin (IVIG) was initiated and followed by a rapid 48-hour increase in the PLT count. PLT antibodies lacking serologic specificity were subsequently identified in a sample collected upon presentation. Two weeks later he again presented to the ED with epistaxis and severe thrombocytopenia (<10 × 10(9)/L). Clinical history now revealed that the patient had been treated with trimethoprim-sulfamethoxazole by his primary care physician after his first hospitalization for a "cellulitic-appearing" leg and again before his final presentation for surgical site erythema and edema. IVIG was administered again with a rapid return of PLT count to baseline. Sulfamethoxazole-dependent PLT antibodies were subsequently identified in the original patient sample. CONCLUSION: This case report documents a case of IVIG-responsive DITP initially misdiagnosed as PTP, highlighting the clinical overlap of these immunologic-mediated phenomena.