The World Health Organization (WHO) classification of the myeloid neoplasms.

Acute monoblastic leukemia: a clinical and biologic study of 74 cases.

BACKGROUND AND OBJECTIVE: The diagnosis of myelodysplastic syndromes (MDS) is essentially morphological and based on the presence of dysplastic features in the peripheral blood and bone marrow. The French-American-British (FAB) Cooperative Group proposed a classification based on easily obtainable laboratory information. In spite of some limitations, the FAB criteria have been useful for a long time. Currently, the recognition of other distinct morphological MDS subgroups such as hypocellular MDS and MDS with myelofibrosis, the increasing incidence of MDS in children as well as that of therapy-related MDS, and the finding of specific chromosomal alterations associated with different morphological features, reveal the insufficiency of this classification. The aim of the present review is to examine some new aspects of the diagnosis, classification, and cytogenetics of MDS. EVIDENCE AND INFORMATION SOURCES: The authors of this review have been actively working and contributing original papers on MDS for the last 15 years. They also organized or participated in the Fourth International Symposium on MDS (Barcelona, April 24-27, 1997). In addition, the present review critically examines relevant articles and abstracts published in journals covered by the Science Citation Index and Medline. STATE OF THE ART AND PERSPECTIVES: Most of investigators working on MDS tend to integrate morphology and cytogenetics in the diagnosis and classification of these disorders. FAB criteria remain useful particularly for patients with not available cytogenetic study. Refractory cytopenia with multilineage dysplasia should be considered as a new MDS subtype. Some authors propose considering all patients with more than 20% of blast cells in peripheral blood or bone marrow as having acute leukemia. Chronic myelomonocytic leukemia with myeloproliferative features may be included among chronic myeloproliferative disorders. MDS with myelofibrosis is recognized as a new MDS subtype. Therapy-related MDS (t-MDS) should be classified according to the involved agents. Finally, besides including chromosomal abnormalities in the diagnosis (e.g., RAEB with trisomy 8), several cytogenetic abnormalities such as deletion 5q and deletion 17q, associated to specific clinical-morphological features, should be of help to identify new MDS syndromes.

/pdf/diagnosis-classification-and-cytogenetics-of-myelodysplastic-4q8lxx0xtw.pdf

Diagnosis, classification, and cytogenetics of myelodysplastic syndromes

Myelofibrosis is characterized by reticulin fibrosis of the bone marrow with resulting features of myelophthisis. Besides hematopoietic malignancies and other neoplasms involving the bone marrow, myelofibrosis has been described in association with autoimmune disorders, especially systemic lupus erythematosus. We describe the clinicopathologic features of a primary form of autoimmune myelofibrosis (AIMF) in patients who do not have systemic lupus erythematosus or another well-defined autoimmune syndrome. Absence of marked splenomegaly, peripheral blood cytopenias with mild teardrop poikilocytosis and leukoerythroblastosis, bone marrow lymphoid aggregates, and presence of autoantibodies are some of the salient features of primary AIMF. AIMF should especially be differentiated from chronic idiopathic myelofibrosis, a neoplastic myeloproliferative disease. Primary AIMF appears to have an excellent prognosis, with all patients reported in this series responding to a short course of corticosteroid therapy.

https://onlinelibrary.wiley.com/doi/pdf/10.1002/ajh.10258

Primary autoimmune myelofibrosis: definition of a distinct clinicopathologic syndrome.

Morphology and classification of the myelodysplastic syndromes and their pathologic variants.

Myelodysplastic syndromes (MDS) and acute myeloid leukemias (AML) with myelofibrosis.

Some patients present borderline features between acute myeloid leukemia (AML) and typical myelodysplastic syndromes (MDS): an excess of blasts insufficient to conclusively diagnose AML, yet above the figures usually compatible with MDS or the presence of Auer rods associated with a moderate excess of blasts. This presents considerable difficulties in diagnosis and management. The authors studied 28 such cases using the French-American-British Co-operative Group (FAB) classification, which groups them into a separate category termed "refractory anemia with excess of blasts in transformation" (RAEB-T). This was found to be a heterogenous group. Certain patients (4/28) had a previously established myelodysplasia, but most presented directly as RAEB-T. Two very different pictures emerged: a few patients (4/28) were young, with presentation and evolution similar to classic AML, for whom combination chemotherapy was effective; the majority (20/28) were older, with more varied clinical and cytologic presentation, for whom chemotherapy was of little effect and who presented a picture resembling classic RAEB with a median survival of 10 months.

Myelodysplastic syndrome or acute myeloid leukemia? A study of 28 cases presenting with borderline features.

Twelve cases of pure acute monocytic leukemia in adults were studied. They were selected on the basis of the morphology of the blast cells on Romanowsky-stained smears of blood and bone marrow, as well as positivity of the cells for the naphthol ASD acetate esterase reaction specifically inhibited by sodium fluoride. There was no sex predominance. Neoplastic involvement of the skin and/or gingiva was very frequent. The leukemic proliferation in blood and bone marrow consisted of monoblasts, promonocytes and monocytes. The peroxidase reaction was negative or only faintly positive. Serum and urinary lysozyme levels were increased. The blast cells retained their ability to stimulate, in vitro , colony formation by normal bone marrow cells used as targets. All of these characteristics permit specific identification of this type of acute leukemia. The prognosis is grim: only five of 12 patients achieved complete remission, and four of these five had relapses in less than 14 months; the median survival was five months.

Pure acute monocytic leukemia. A study of 12 cases.

Manual of Hematology is designed in outline form to provide a concise and clear overview of the subject of hematology. Both laboratory and clinical aspects of hematologic disorders are covered, but the primary emphasis is on the pathophysiology of hematologic disorders. The book is divided into 5 sections. Part 1 provides a concise review of the structure and function of the normal cell components of the hemic system as well as the major plasma components, such as iron, vitamin B12 and folate, that are of special interest in hematologic disease. Part 2 deals with the major disease groups and presents the important basic features of the disorders. Part 3 deals with hemostasis and coagulation. Part 4 covers immunohematology, with special attention to the blood group antigens, HLA antigens, use of blood components in therapy and transfusion reactions. The last section of the book consists of a series of diagnostic problems in which pertinent clinical and laboratory data are provided. These cases are selected to teach interpretation of laboratory data in a logical manner and should prove a valuable and interesting aspect of the book.

Manual of hematology

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Myelodysplastic syndromes (MDS) and acute myeloid leukemias (AML) with myelofibrosis.

Myelodysplastic syndrome or acute myeloid leukemia? A study of 28 cases presenting with borderline features.

Pure acute monocytic leukemia. A study of 12 cases.

Manual of hematology