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PubMed1999

Hypercalcemia complicating leukemic transformation of agnogenic myeloid metaplasia-myelofibrosis.

Hypercalcemia is a common and potentially life-threatening metabolic derangement associated with many malignancies, especially solid tumors and multiple myeloma. Hypercalcemia has been reported only rarely with acute myelogenous leukemia. We describe a patient who developed hypercalcemia in association with transformation of agnogenic myeloid metaplasia into M7 acute myelogenous leukemia. Laboratory investigation revealed low levels of serum parathyroid hormone, undetectable levels of parathyroid hormone-related peptide, and normal levels of 25-hydroxyvitamin D. These observations suggest that another mediator was responsible for hypercalcemia in this patient. Awareness of this rare complication of acute myelogenous leukemia is essential for prompt diagnosis and management.

Cell Transformation, Neoplastic↗
PubMed1955

Myelofibrosis.

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Arteriosclerosis↗
PubMed1995

Hairy cell leukemia without splenomegaly nor myelofibrosis in a patient with gastric adenocarcinoma: early phase of the disease or a variant?

The first case of patient affected both by gastric carcinoma and hairy cell leukemia (HCL) is reported. From a clinical standpoint, this 54-year old man presented with striking leukopenia without splenomegaly. From a morphological point of view, the infiltration by leukemic cells occurred in the hypoplasic areas of the bone marrow biopsy (BMB) without increase in reticulin fibers. From these observations the authors deduce that: 1) BMB in many cases is the only morphologic tool for diagnosis and prognosis of HCL; 2) probably the "hypoplasic" variant of HCL will become more frequent, because of the increasing indication to BMB in course of pancytopenia, even in absence of splenomegaly; 3) our case is probably related more to an early phase of the disease than to a distinct variant. In addition, we propose that the co-existence of an aggressive solid tumor and HCL could be related to the immunosuppressive action by hairy cells.

Adenocarcinoma↗
PubMed2005

De novo appearance of der(1;7)(q10;p10) is associated with leukemic transformation and unfavorable prognosis in essential thrombocythemia.

Leukemic transformation or myelofibrosis is a major concern in managing patients with chronic myeloproliferative disorders, including essential thrombocythemia (ET). We analyze the relationship between cytogenetic changes and the transformation in 89 patients with ET; 8 patients experienced transformation, including 2 patients with acute leukemia following myelofibrosis, 3 with acute leukemia, and 3 with myelofibrosis. Among the eight patients showing transformation, two patients developing myelofibrosis derived from a group with normal cytogenetics, but the remaining six were categorized as showing de novo appearance of cytogenetic changes. Two leukemia patients had de novo cytogenetic changes at the time of leukemia diagnosis, whereas two patients with acute leukemia following myelofibrosis showed der(1;7) during their myelofibrosis period. Moreover, patients with der(1;7) did not receive any cytotoxic agents before the appearance of der(1;7), indicating that detection of der(1;7) may have predictive value for not only leukemic transformation but also unfavorable prognosis.

Acute Disease↗
PubMed1997

Clinicopathological impact of the interaction between megakaryocytes and myeloid stroma in chronic myeloproliferative disorders: a concise update.

In this report an attempt has been made to discuss some of the issues pertinent to myelofibrosis complicating chronic myeloproliferative disorders (CMPDs) that are significantly associated with megakaryocyte function. In this context, biochemical, clinical and particularly morphological features were reviewed. Morphological findings based on elaborate techniques were in keeping with the assumption that in chronic myeloid leukemia (1) the number of CD61-positive megakaryocytes, and in particular their precursors were the parameters most closely associated with myelofibrosis (2) an increased content of reticulin fibers in follow-up biopsies significantly correlated with laboratory data indicative of a high tumor burden (anemia, peripheral blasts, hepatosplenomegaly) and thus a more advanced stage of the disease process (3) even a slight increase in reticulin, i.e. doubling of the normal fiber density was associated with a worse prognosis independent of therapeutic regimens given (4) Dynamics of myelofibrosis was significantly influenced by treatment. In this context, calculation of the myelofibrosis progression index (MPI) revealed a higher score following interferon therapy compared with busulfan. In addition, in idiopathic myelofibrosis (5) the evolution of myelofibrosis was unpredictable and according to the MPI, progression occurred at a relatively low rate (6) proliferation and dilatation of sinusoids accompanying intravascular hematopoiesis and collagen type IV deposits were predominant features in later (fibro-osteosclerotic) stages in the course of disease (7) transmural migration of megakaryocytes demonstrated by three dimensional reconstruction revealed a mole-like tunneling through the thickened sinusoidal wall. A very careful assessment of the numerous correlations between bone marrow features and laboratory data will allow clinicians and pathologists to gain a better insight into the mutual relationships between hematological and morphological findings in CMPDs.

Cell Communication↗
PubMed1983

Megakaryocytes in cubital venous blood in patients with chronic myeloproliferative diseases.

33 patients with chronic myelo-proliferative disease were investigated for megakaryocytes in cubital venous blood. 6 Patients had chronic myeloid leukaemia, 11 polycythaemia vera and 16 myelofibrosis. In all patients, a significantly higher number of megakaryocytes than in normal adult humans was found. In patients with chronic myeloid leukaemia and myelofibrosis, an increased number of intact megakaryocytes was found, indicating thrombopoiesis in the peripheral part of the upper extremity. 2 patients with myelofibrosis showed a % of intact megakaryocytes greater than 25, as found in blood from the inferior vena cava in normal adults. In patients with chronic myeloid leukaemia, some of the megakaryocytes were smaller than normal, whereas in myelofibrosis, some of the megakaryocytes were larger than normal, with increased lobulation of nuclei. Active thrombopoiesis in the peripheral part of the extremities can be of great importance, when splenectomy is considered in treatment of myelofibrosis.

Adult↗
PubMedDate not supplied

Comparative effects of interferon and hydroxyurea on bone marrow fibrosis in chronic myelogenous leukemia.

Therapy-related changes of the bone marrow fiber content remain a controversial issue in hematopathology. This conflict of opinion firstly depends on difficulties to determine the quantity of fibers exactly (semiquantitative grading, morphometry, reference to cellularity). Secondly, the appropriate selection of patients with specific monotherapies including hydroxyurea (HU) and interferon-alpha (IFN) seems to present some problems. Finally, assessment of myelofibrosis is further biased by the different endpoints of sequential examinations. The latter shortcoming can be improved upon by the calculation of the myelofibrosis progression/regression index which describes the ratio between difference of fiber density and observation time. Using strictly defined therapeutic regimens and intervals between sequential trephine biopsies a stimulating effect of IFN administration on bone marrow fibrosis in Ph1+-chronic myelogenous leukemia (CML) has been found. This result is comparable with the failure of this agent to improve myelofibrosis (and splenomegaly) in a considerable number of patients with allied subtypes of chronic myeloproliferative disorders. This is in contrast to the effect HU exerts which is a more fibrolytic or even stabilizing influence on bone marrow fibrosis. This phenomenon is readily demonstrable by the assessment of dynamic features (myelofibrosis progression index). In addition, patients showing a rapid progression of myelofibrosis during IFN and HU treatment of Ph1+-CML are generally associated with a poor risk outcome and a significant worsening of survival.

Humans↗
PubMedDate not supplied

[Bone marrow fibrosis in malignant hemopathies and cancers. Histological study of 2 786 biopsies (author's transl)].

This is a study of myelofibrosis concerning 2.786 bone marrow biopsies in malignant hemopathies, and cancers supposed to be metastatic. In a first group myelofibrosis is a permanent symptom and necessary for the diagnosis: agnogenic myeloid metaplasia, Waldenström's disease, hairy-cell leukemia, marrow extension of Hodgkin's disease and metastatic cancers. In a second group of hemopathies, myelofibrosis varies in frequency. Myeloid leukemia: 33% - Polycythemia: 40% - Thrombocythemia: 27% - Lymphoid leukemia: 17% - Acute leukemia: 20% - Kahler's disease: 30% - Aplastic anemia: 5% - Reticulin myelofibrosis is said possibly regressive. Collagen myelofibrosis is permanent.

Acute Disease↗
PubMed1995

Karyotype findings and molecular analysis of the bcr gene rearrangement supplementing the histologic classification of chronic myeloproliferative disorders.

BACKGROUND: Histologic examination of bone marrow is important in establishing diagnoses among chronic myeloproliferative disorders (CMPD). Only a few studies, however, have compared cytogenetic or molecular genetic findings to histopathology in CMPD. Diverging results on the presence of the Ph1-translocation in patients with myelofibrosis have been reported. EXPERIMENTAL DESIGN: Cytogenetic studies and molecular analysis of the bcr gene were performed in bone marrow cells of patients with CMPD simultaneously with histopathologic examination of plastic-embedded bone marrow biopsies. RESULTS: The Ph1-chromosome was found in 120/128 (93%) cases with histopathologic diagnosis of chronic myeloid leukemia (CML), including a notable proportion of cases with an increase of megakaryocytes and/or myelofibrosis; the latter was associated with a significant increase of chromosome aberrations, in addition to Ph1. Among those additional changes in myelofibrosis of Ph1-positive CML were del (13q) and t(1;11) in one case each. A bcr gene rearrangement was detected in 92% (24/26) of the CML cases examined. All other groups of CMPD, comprising cases of myelofibrosis and unclassifiable cases, were Ph1-negative by both cytogenetics (n = 102) and molecular analysis (n = 18). Karyotype changes associated with myelofibrosis in various CMPD concerned mainly balanced translocations involving 1p36 and 11q11, deletions of 5q13-34, 3p, 11q23, 13(q12,q22), and 20q12 as well as gain of 1q and trisomy 3, 8, 19, or 21. In histologically unclassifiable CMPD, karyotyping provided additional information for the differential diagnosis. CONCLUSIONS: The correlation of cytogenetic findings and histopathologic features is helpful in confirming or supporting histopathologic diagnoses and in characterizing new marker chromosomes in CMPD.

Aged↗