scholarly journals Leukemias with megakaryoblastic involvement: clinical, hematologic, and immunologic characteristics

Blood ◽  
1988 ◽  
Vol 72 (2) ◽  
pp. 402-407
Author(s):  
JF San Miguel ◽  
M Gonzalez ◽  
MC Canizo ◽  
E Ojeda ◽  
A Orfao ◽  
...  
Keyword(s):  
Poor Prognosis ◽  
De Novo ◽  
Myeloproliferative Disorder ◽  
Lower Percentage ◽  
Severe Anemia ◽  
Bone Marrow Fibrosis ◽  
Entire Series ◽  
Hla Dr ◽  
Marrow Fibrosis ◽  
Secondary Leukemias

The clinical, hematologic, and phenotypic features of 28 patients with acute leukemia with megakaryocytic involvement (AMKL) were analyzed. The prevalence of this type of leukemia in the entire series was 11.6%, with a higher incidence among patients with acute transformation of a previous myeloproliferative disorder (MPD) (24%) than among the transformed myelodysplastic syndrome (13%) patients. The incidence in the “de novo” ANLL was 8% and 16% among secondary leukemias. The presence of bone marrow fibrosis together with low WBC and normal or increased platelet counts despite a severe anemia are the most relevant features in these patients who otherwise displayed an apparently poor prognosis. Megakaryoblasts were morphologically recognized more frequently in the acute transformations of MPD than in de novo ANLL. Only two cases were considered pure AMKL, and in the remaining 26 patients, megakaryoblasts coexisted with other granulomonocytic and/or erythroid populations. Antiglycoprotein IIIa (anti-GPIIIa) (C17) and anti-GPIIb/IIIa (CDw41-, J15-) antibodies are probably the best markers for AMKL, although the monoclonal antibody against GPIX (FMC25) was also positive in a majority of cases but in a lower percentage of cells. On the other hand, megakaryoblasts were generally negative for granulocytic or monocytic markers (CD13, CD14, CD15); the expression of HLA-DR antigens in these cells was variable. Our present results indicate that megakaryoblastic involvement is more common than previously recognized. This is true not only in acute transformed leukemias but also in de novo ANLL. Although the diagnosis of these cases should be based on megakaryocytic markers, it is often possible to suspect a diagnosis according to certain clinical and hematologic features.

scholarly journals Leukemias with megakaryoblastic involvement: clinical, hematologic, and immunologic characteristics

Blood ◽  
1988 ◽  
Vol 72 (2) ◽  
pp. 402-407 ◽  
Author(s):  
JF San Miguel ◽  
M Gonzalez ◽  
MC Canizo ◽  
E Ojeda ◽  
A Orfao ◽  
...  
Keyword(s):  
Poor Prognosis ◽  
De Novo ◽  
Myeloproliferative Disorder ◽  
Lower Percentage ◽  
Severe Anemia ◽  
Bone Marrow Fibrosis ◽  
Entire Series ◽  
Hla Dr ◽  
Marrow Fibrosis ◽  
Secondary Leukemias

Abstract The clinical, hematologic, and phenotypic features of 28 patients with acute leukemia with megakaryocytic involvement (AMKL) were analyzed. The prevalence of this type of leukemia in the entire series was 11.6%, with a higher incidence among patients with acute transformation of a previous myeloproliferative disorder (MPD) (24%) than among the transformed myelodysplastic syndrome (13%) patients. The incidence in the “de novo” ANLL was 8% and 16% among secondary leukemias. The presence of bone marrow fibrosis together with low WBC and normal or increased platelet counts despite a severe anemia are the most relevant features in these patients who otherwise displayed an apparently poor prognosis. Megakaryoblasts were morphologically recognized more frequently in the acute transformations of MPD than in de novo ANLL. Only two cases were considered pure AMKL, and in the remaining 26 patients, megakaryoblasts coexisted with other granulomonocytic and/or erythroid populations. Antiglycoprotein IIIa (anti-GPIIIa) (C17) and anti-GPIIb/IIIa (CDw41-, J15-) antibodies are probably the best markers for AMKL, although the monoclonal antibody against GPIX (FMC25) was also positive in a majority of cases but in a lower percentage of cells. On the other hand, megakaryoblasts were generally negative for granulocytic or monocytic markers (CD13, CD14, CD15); the expression of HLA-DR antigens in these cells was variable. Our present results indicate that megakaryoblastic involvement is more common than previously recognized. This is true not only in acute transformed leukemias but also in de novo ANLL. Although the diagnosis of these cases should be based on megakaryocytic markers, it is often possible to suspect a diagnosis according to certain clinical and hematologic features.

scholarly journals Idiopathic myelofibrosis mimicking hemolytic anemia

2012 ◽  
Vol 2 (4) ◽  
pp. 324-327
Author(s):  
R Baral ◽  
G Aryal ◽  
KC Shiva Raj
Keyword(s):  
Bone Marrow ◽  
Clinical Course ◽  
Myeloproliferative Disorder ◽  
Severe Anemia ◽  
Idiopathic Myelofibrosis ◽  
Blood Picture ◽  
Bone Marrow Fibrosis ◽  
Hematopoietic Stem ◽  
Chronic Myeloproliferative Disorder ◽  
Marrow Fibrosis

Idiopathic Myelofibrosis is an infrequent chronic myeloproliferative disorder characterized by varying degrees of bone marrow fibrosis and extra medullary hematopoiesis, with the fibrosis being a reactive phenomenon to a neoplastic proliferation of a pluripotent hematopoietic stem cell. Idiopathic Myelofibrosis is heterogeneous in presentation and clinical course, with anemia being one of the most important problems. We present a case of a 59 year old male who presented with severe anemia, the peripheral blood picture mimicking hemolysis with numerous schistocytes and teardrop cells.Journal of Pathology of Nepal (2012) Vol. 2, 323-327DOI: http://dx.doi.org/10.3126/jpn.v2i4.6888

The presence of bone marrow fibrosis is associated with poorer prognosis in newly diagnosed multiple myeloma treated with immunomodulatory agents and/or proteasome inhibitors.

2019 ◽  
Vol 37 (15_suppl) ◽  
pp. e19514-e19514
Author(s):  
Barry Paul ◽  
Gavin Loitsch ◽  
Daniel Feinberg ◽  
Ian Barak ◽  
Zhiguo Li ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Bone Marrow ◽  
Poor Prognosis ◽  
Proteasome Inhibitors ◽  
Cell Transplant ◽  
Newly Diagnosed ◽  
Bone Marrow Fibrosis ◽  
Bone Marrow Biopsies ◽  
Immunomodulatory Agents ◽  
Marrow Fibrosis

e19514 Background: The treatment of newly diagnosed multiple myeloma (NDMM) has evolved significantly with the advent of the immunomodulatory agents (IMiDs) and proteasome inhibitors (PIs). While the presence of bone marrow fibrosis (BMF) has previously been associated with poor prognosis in multiple myeloma (MM), these studies were small and conducted prior to the widespread use of IMiDs and PIs. Here, we determined the incidence of BMF in NDMM patients and correlated the degree of BMF with prognosis in a population enriched for IMiD and/or PI exposure. Methods: Bone marrow biopsies from 306 MM patients seen at Duke between 2003 and 2013 were screened for BMF using a reticulin stain. Samples were scored as absent, mild, moderate, or severe fibrosis based on the degree and intensity of staining. The association between presence and degree of BMF to progression free survival (PFS) and overall survival (OS) was calculated using Kaplan-Meier analysis. Results: Of the 306 patients evaluated, 248 (81.0%) were treated with an IMiD, 241 (78.8%) were treated with a PI, and 217 (70.9%) received both. Additionally, 160 (52.3%) patients went on to receive an autologous stem cell transplant (HSCT). A total of 193 patients (63.1%) were evaluable for BMF. Of these, 96 (49.7%) had detectable BMF, while 97 (50.3%) had no BMF. The degree of BMF was mild in 60 patients (62.5%), and moderate or severe in 34 patients (35.4%). Median PFS in patients without BMF was 30.4 months, and 21.8 months in patients with BMF present (log-rank p = 0.02). Median OS was 61.1 months in patients without BMF, and 46.3 months in patients with BMF (log-rank p = 0.048). Patients with moderate or severe BMF had a particularly poor prognosis with a PFS of only 18.8 months and an OS of 32.7 months. Conclusions: Our study represents the largest dataset to date examining the incidence of BMF in MM patients, and is the only one to examine the association of BMF with prognosis in the era of novel therapies and widespread use of HSCT. Our data suggests that BMF is common in NDMM, and MM patients with BMF (particularly those with more extensive BMF) have a poorer prognosis even when treated with IMiDs and PIs. These data emphasize the importance of determining the presence and degree of BMF at time of MM diagnosis, and suggest a role for adjunctive therapies that target BMF in MM patients with co-existing BMF.

Essential Thrombocythemia: A Review of Diagnostic and Pathologic Features

2006 ◽  
Vol 130 (8) ◽  
pp. 1144-1150
Author(s):  
Steven Sanchez ◽  
April Ewton
Keyword(s):  
Essential Thrombocythemia ◽  
Myeloproliferative Disorder ◽  
Janus Kinase ◽  
Data Sources ◽  
Bone Marrow Fibrosis ◽  
Jak2 Mutation ◽  
Pathologic Features ◽  
Laboratory Features ◽  
Chronic Myeloproliferative Disorder ◽  
Marrow Fibrosis

Abstract Context.—Essential thrombocythemia (ET) is a chronic myeloproliferative disorder (CMPD) characterized predominately by thrombocytosis and abnormal megakaryocyte proliferation. The current diagnostic criteria require a combination of clinical, histologic, and cytogenetic data. The diagnosis relies largely on exclusion of other causes of thrombocytosis. Objective.—Describe historical, clinical, and laboratory features of ET in order to understand, clarify, and more accurately diagnose this entity. Data Sources.—Review contemporary and historical literature on ET and other causes of thrombocytosis. Conclusions.—ET is a relatively indolent and often asymptomatic CMPD that is characterized primarily by a sustained elevation in platelets ≥600 × 103/μL (≥600 × 109/L), proliferating enlarged and hyperlobated megakaryocytes, and minimal to absent bone marrow fibrosis. Significant changes and revisions to the diagnostic requirements and criteria for ET have occurred during the last 30 years. Recently, a mutation in the Janus kinase 2 (JAK2) gene has been found in a significant number of cases of ET and other CMPDs. In up to 57% of ET cases, a mutation in the JAK2 gene can be detected. In the absence of a JAK2 mutation and features of another CMPD, the diagnosis of ET remains a diagnosis of exclusion after other causes of thrombocytosis have been excluded.

scholarly journals Disseminated tuberculosis with myelofibrosis presentation: a case report

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Mahdi Khatuni ◽  
Marziyeh Ghalamkari ◽  
Fereshteh Ameli ◽  
Habibeh Yekehtaz
Keyword(s):  
Bone Marrow ◽  
Primary Myelofibrosis ◽  
Final Diagnosis ◽  
Myeloproliferative Disorder ◽  
Middle Aged ◽  
Bone Marrow Fibrosis ◽  
Case Presentation ◽  
Old Adults ◽  
Disseminated Tuberculosis ◽  
Marrow Fibrosis

Abstract Background Primary myelofibrosis is a rare myeloproliferative disorder in middle-aged and old adults and should be distinguished from secondary and reactive causes of bone marrow fibrosis because, in reactive fibrosis, treatment approaches depend on the underlying etiology. Case presentation Here we report the case of a middle-aged Iranian man who was diagnosed and treated as primary myelofibrosis at presentation, and whose final diagnosis was disseminated tuberculosis with reactive bone marrow fibrosis. Conclusions It is prudent to evaluate the potential causes of myelofibrosis in any patient with the diagnosis primary myelofibrosis. Tuberculosis can be an important etiology of bone marrow fibrosis, especially in endemic areas.

scholarly journals Prolonged Myelosuppression due to Progressive Bone Marrow Fibrosis in a Patient with Acute Promyelocytic Leukemia

2019 ◽  
Vol 2019 ◽  
pp. 1-7
Author(s):  
Yuta Inagawa ◽  
Yukiko Komeno ◽  
Satoshi Saito ◽  
Yuji Maenohara ◽  
Tetsuro Yamagishi ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Acute Promyelocytic Leukemia ◽  
Bone Marrow Biopsy ◽  
Gastric Fluid ◽  
Promyelocytic Leukemia ◽  
Consolidation Therapy ◽  
Bone Marrow Fibrosis ◽  
All Trans Retinoic Acid ◽  
Phlegmonous Gastritis ◽  
Marrow Fibrosis

A 34-year-old woman was diagnosed with acute promyelocytic leukemia. Chemotherapy was administered following the JALSG APL204 protocol. Induction therapy with all-trans retinoic acid resulted in complete remission on day 49. She developed coccygeal pain from day 18, which spread to the spine and cheekbones and lasted 5 weeks. She had similar bone pain on days 7–10 of the first consolidation therapy and on days 4–12 of the second consolidation therapy. Oral loxoprofen was prescribed for pain relief. On day 33 of the third consolidation, white blood cell and neutrophil counts were 320/μL and 20/μL, respectively. After she developed epigastralgia and hematemesis, she developed septic shock. Gastroendoscopy revealed markedly thickened folds and diffusely damaged mucosa with blood oozing. Computed tomography revealed thickened walls of the antrum and the pylorus. Despite emergency treatments, she died. Bacterial culture of the gastric fluid yielded Enterobacter cloacae and enterococci growth. Collectively, she was diagnosed with phlegmonous gastritis. Retrospective examination of serial bone marrow biopsy specimens demonstrated progressive bone marrow fibrosis, which may have caused prolonged myelosuppression. Thus, evaluation of bone marrow fibrosis by bone marrow biopsy after each treatment cycle might serve as a predictor of persistent myelosuppression induced by chemotherapy.

Metabolism of [3-13C]alanine in liver of mice infected with cysticerci of Taenia crassiceps

1999 ◽  
Vol 77 (9) ◽  
pp. 1367-1372 ◽  
Author(s):  
I Corbin ◽  
B J Blackburn ◽  
M Novak
Keyword(s):  
Nuclear Magnetic Resonance ◽  
Nmr Spectroscopy ◽  
Population Inversion ◽  
De Novo ◽  
Spin Echo ◽  
Proton Spin ◽  
Lower Percentage ◽  
Nmr Analysis ◽  
Taenia Crassiceps ◽  
Hepatic Glucose

Carbon-13-decoupled proton spin-echo nuclear magnetic resonance (NMR) spectroscopy, with and without 13C population inversion, was used to study carbon flow between the host and the parasite in the mouse - Taenia crassiceps system. This NMR analysis revealed that 2 h after intraduodenal injection of [3-13C]alanine, livers from both uninfected mice and those infected with cysticerci of T. crassiceps contained 13C label in glycogen, glucose, succinate, glutamate, alanine, and lactate. Livers of infected animals had a lower percentage of 13C in alanine, indicating increased utilization of the substrate. In addition, infected mice had a lower concentration of total hepatic glucose and glutamate. The data are consistent with an increased rate of gluconeogenesis in the liver of infected animals. Cysticerci possessed 13C label in glucose, acetate, alanine, and lactate. Since these metacestodes are unable to make glucose de novo from pyruvate, labelled glucose found in cysticerci had to be newly synthesized via the host gluconeogenic pathway and then siphoned off by the parasite.

Sign in / Sign up

Export Citation Format

Share Document