Acute Panmyelosis with Myelofibrosis - A Rare Subtype of Acute Myeloid Leukemia

Chatterjee, Tathagata; Gupta, Srishti; Sharma, Ajay; Sharma, Sanjeevan; Gupta, Devika

doi:10.4084/mjhid.2013.042

Cited by 8 publications

(5 citation statements)

References 8 publications

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“…Although MF patients are not typically CD33 positive, acute panmyelosis with MF, a specific subtype of MF, has been found to display this cellular marker. 59 Additionally, other studies on MF patients have found that spleens of MF patients can contain populations of CD33 malignant hematopoietic stem cells. 60 This suggests that, for those who are not candidates for intensive therapy or who do not have alternative treatment options and are CD33 positive, gemtuzumab should be considered.…”

Section: Initial Blast-phase Treatment Optionsmentioning

confidence: 99%

Managing myelofibrosis (MF) that “blasts” through: advancements in the treatment of relapsed/refractory and blast-phase MF

Scherber

Mesa

2018

Hematology

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Myelofibrosis (MF) is the most aggressive form of Philadelphia chromosome–negative myeloproliferative neoplasm, and it is complicated by severe symptom burden, thrombotic events, infections, cytopenias, and transformation to acute myeloid leukemia (AML). Ruxolitinib, the first-line therapy for symptomatic or intermediate- and high–prognostic risk MF, has improved overall survival for this population. However, approximately one-half of MF patients will discontinue ruxolitinib by the first few years of therapy due to a spectrum of resistance, intolerance, relapse, or progression to blast phase disease. Danazol, erythropoietin-stimulating agents, and spleen-directed therapies can be useful in the ruxolitinib-resistant setting. In the ruxolitinib-refractory or -intolerant setting, commercial and novel therapies, either alone or in combination with ruxolitinib, have shown clinical utility. For blast-phase MF, the recent advancements in available AML therapies have increased the options with targeted and more tolerable therapies. In this article, we will discuss our paradigm for the management of relapsed/refractory and blast-phase MF in the context of therapeutic advancements in both AML and MF.

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Section: Initial Blast-phase Treatment Optionsmentioning

confidence: 99%

Managing myelofibrosis (MF) that “blasts” through: advancements in the treatment of relapsed/refractory and blast-phase MF

Scherber

Mesa

2018

Hematology

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“…APMF usually presents with pancytopenia in the peripheral blood with absent or only rare circulating blasts without overt anisopoikilocytosis [ 20 ]. Splenomegaly is absent, and extramedullary hematopoiesis in the spleen is not prominent [ 21 ]. The clinical course of APMF is rapidly progressive and fatal often terminating with an overt acute leukemia, therefore, it is essential to make the accurate diagnosis and distinguish it from its mimickers, particularly acute megakaryoblastic leukemia (AML-M7) through a detailed clinical history and hematological work up [ 20 , 22 ].…”

Section: Resultsmentioning

confidence: 99%

Acute Myeloid Leukemia with Isolated Trisomy 19 Associated with Diffuse Myelofibrosis and Osteosclerosis

Stelling¹,

Jonas²,

Rashidi³

et al. 2015

Cancers

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Primary myelofibrosis (PMF), per WHO criteria, is a clonal myeloproliferative neoplasm that usually presents with a proliferation of granulocytic and megakaryocytic lineages with an associated fibrous deposition and extramedullary hematopoiesis. The bone marrow histologic findings of this disorder are typically characterized by the presence of myeloid metaplasia with an associated reactive fibrosis, angiogenesis, and osteosclerosis. However, marked myelofibrosis is not solely confined to PMF and may also be associated with other conditions including but not limited to acute megakaryoblastic leukemias (FAB AML-M7). Here, we describe a rare case of a non-megakaryoblastic acute myeloid leukemia with marked myelofibrosis with osteosclerosis and an isolated trisomy 19. A 19-year-old male presented with severe bone pain of one week duration with a complete blood cell count and peripheral smear showing a mild anemia and occasional circulating blasts. A follow up computed tomography (CT) scan showed diffuse osteosclerosis with no evidence of hepatosplenomegaly or lymphadenopathy. Subsequently, the bone marrow biopsy showed markedly sclerotic bony trabeculae and a hypercellular marrow with marked fibrosis and intervening sheets of immature myeloid cells consistent with myeloblasts with monocytic differentiation. Importantly, these myeloblasts were negative for megakaryocytic markers (CD61 and vWF), erythroid markers (hemoglobin and E-cadherin), and lymphoid markers (CD3, CD19, and TdT). Metaphase cytogenetics showed an isolated triosomy 19 with no JAK2 V617F mutation. The patient was treated with induction chemotherapy followed by allogenic hematopoietic stem cell transplantation which subsequently resulted in a rapid resolution of bone marrow fibrosis, suggesting graft-anti-fibrosis effect. This is a rare case of a non-megakaryoblastic acute myeloid leukemia with myelofibrosis and osteosclerosis with trisomy 19 that may provide insights into the prognosis and therapeutic options of future cases.

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“…Follow-up revealed that immunohistochemistry was not contributing towards a definite diagnosis and the patient was treated as a case of acute myeloid leukaemia and there was improvement in symptoms. Many published articles consider APMF as a variant of myelodysplastic syndrome [ 13 ]. It is important to have knowledge about this entity since these patients need aggressive management due to the rapidly fatal course and poor outcome associated with this condition.…”

Section: Discussionmentioning

confidence: 99%

Utility of Bone Marrow Examination in Pediatric Age Group: Experience of a Tertiary Healthcare Centre in India

Meenakshi¹,

Sangoi²

2022

Cureus

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Bone marrow is involved in various haematological and non-haematological conditions in the paediatric age group. To understand and analyse the underlying etiology, bone marrow examination, including both bone marrow aspiration and trephine biopsy, forms an important diagnostic tool. It helps to differentiate proliferative disorders of the myeloid and lymphoid series, find out their prognosis based on severity, and assess their status before therapy is started. It also aids in staging and in the detection of infiltration of the marrow by foreign cells. The spectrum of disorders affecting the marrow in children ranges from common disorders like nutritional deficiency anaemia to serious conditions like leukaemia. Their line of management, severity, and prognosis differ from the bone marrow lesions found in adults. The present study was done to demonstrate the utility of bone marrow examination in the paediatric age group, to demonstrate its contribution to the final diagnosis of the patient, and to list and study the various lesions encountered in this age group in a tertiary care teaching hospital and for better understanding of clinicopathological features and microscopy, suggesting variation with or convergence with literature. Material and methodsThis was an observational study with a combination of retrospective and prospective studies, carried out over a period of six years, from January 2014 to December 2019. All bone marrow aspiration and biopsy samples received in the surgical pathology department of our tertiary healthcare centre during the study period of patients between zero and 18 years of age were included. Samples obtained at autopsy and samples where the tissue was diluted or the marrow was inadequate were excluded from the study. Detailed histomorphology was studied in all cases, and the data obtained was recorded in an excel sheet. The descriptive statistics were expressed in terms of numbers and percentages. The study was ethically approved by the Ethics and Research Committee of our hospital before the commencement of the study. ResultsThe maximum number of marrows were performed in the age group of 12-18 years, followed by six to 12 years. The most common indication for performing bone marrow examination was 'evaluation of cytopenias', followed by 'suspected/to rule out haematological malignancy'. A male predominance was found. Overall and amongst neoplastic lesions, acute leukaemia was the commonest diagnosis in the bone marrow. Infections were the second most common lesion overall and the commonest non-neoplastic lesion encountered. ConclusionThe spectrum of lesions requiring bone marrow examination in the paediatric age group is of varied etiologies. Bone marrow examination in children is useful in understanding the pathogenesis, establishing a tissue diagnosis, ruling out haematological malignancies and other causes of cytopenias, planning further lines of investigations and management, and knowing the response to therapy and prognosis.

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Acute Panmyelosis with Myelofibrosis - A Rare Subtype of Acute Myeloid Leukemia

Cited by 8 publications

References 8 publications

Managing myelofibrosis (MF) that “blasts” through: advancements in the treatment of relapsed/refractory and blast-phase MF

Managing myelofibrosis (MF) that “blasts” through: advancements in the treatment of relapsed/refractory and blast-phase MF

Acute Myeloid Leukemia with Isolated Trisomy 19 Associated with Diffuse Myelofibrosis and Osteosclerosis

Utility of Bone Marrow Examination in Pediatric Age Group: Experience of a Tertiary Healthcare Centre in India

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