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Congenital erythroleukemia. A case report.

The disease of a four week old boy is described, which presented with papulous skin infiltrations, lethargy, anemia and bilateral testicular swelling. The investigation of bone marrow, cerebrospinal fluid and skin biopsy revealed infiltration by a leukemic process which could be identified by means of cytology and cytochemistry as erythroleukemia at the stage of erythremic myelosis. Death occurred 20 days after the onset of symptoms. Only one previous report of congenital erythroleukemia could be found in the literature. The findings are briefly discussed.

Biopsy↗

Three or four copies of a dicentric 17q isochromosome in an acute myeloproliferative disorder.

The majority of metaphases in the bone marrow of a male patient aged 72 with a rapidly evolving aleukemic erythremic myelosis had 48 chromosomes with three copies, or 49 chromosomes with four copies, of an i(17q), which was seen to be dicentric in C-banded and Giemsa-11-banded preparations. There was also loss of a chromosome No. 5 and the addition of a chromosome resembling a No. 22. The presence of multiple copies of the isochromosome is postulated to be related to the acuteness of the condition.

Acute Disease↗

Myeloproliferative disease in a calf.

A myeloproliferative disease was found in a 128-day-old Holstein female calf. The tumour consisted chiefly of primitive cells, and more mature cells, many of which were positive for haemoglobin, were admixed with them. Binucleated cells at various stages of maturation were occasionally seen, and siderosomes were confirmed in both primitive cells and more mature cells by electron microscopy. The primitive cells resembled proerythroblasts, and this tumour, thought to be an acute type neoplasm of the erythroid system, was distinguishable from chronic erythremic myelosis and polycythemia vera.

Animals↗

Proposed criteria for classification of acute myeloid leukemia in dogs and cats.

Blood and bone marrow smears from 49 dogs and cats, believed to have myeloproliferative disorders (MPD), were examined by a panel of 10 clinical pathologists to develop proposals for classification of acute myeloid leukemia (AML) in these species. French-American-British (FAB) group and National Cancer Institute (NCI) workshop definitions and criteria developed for classification of AML in humans were adapted. Major modifications entailed revision of definitions of blast cells as applied to the dog and cat, broadening the scope of leukemia classification, and making provisions for differentiating erythremic myelosis and undifferentiated MPD. A consensus cytomorphologic diagnosis was reached in 39 (79.6%) cases comprising 26 of AML, 10 of myelodysplastic syndrome (MDS), and 3 of acute lymphoblastic leukemia (ALL). Diagnostic concordance for these diseases varied from 60 to 81% (mean 73.3 +/- 7.1%) and interobserver agreement ranged from 51.3 to 84.6% (mean 73.1 +/- 9.3%). Various subtypes of AML identified included Ml, M2, M4, M5a, M5b, and M6. Acute undifferentiated leukemia (AUL) was recognized as a specific entity. M3 was not encountered, but this subclass was retained as a diagnostic possibility. The designations M6Er and MDS-Er were introduced where the suffix "Er" indicated preponderance of erythroid component. Chief hematologic abnormalities included circulating blast cells in 98% of the cases, with 36.7% cases having >30% blast cells, and thrombocytopenia and anemia in approximately 86 to 88% of the cases. Bone marrow examination revealed panmyeloid dysplastic changes, particularly variable numbers of megaloblastoid rubriblasts and rubricytes in all AML subtypes and increased numbers of eosinophils in MDS. Cytochemical patterns of neutrophilic markers were evident in most cases of Ml and M2, while monocytic markers were primarily seen in M5a and M5b cases. It is proposed that well-prepared, Romanowsky-stained blood and bone marrow smears should be examined to determine blast cell types and percentages for cytomorphologic diagnosis of AML. Carefully selected areas of stained films presenting adequate cellular details should be used to count a minimum of 200 cells. In cases with borderline diagnosis, at least 500 cells should be counted. The identity of blast cells should be ascertained using appropriate cytochemical markers of neutrophilic, monocytic, and megakaryocytic differentiation. A blast cell count of > 30% in blood and/or bone marrow indicates AML or AUL, while a count of < 30% blasts in bone marrow suggests MDS, chronic myeloid leukemias, or even a leukemoid reaction. Myeloblasts, monoblasts, and megakaryoblasts comprise the blast cell count. The FAB approach with additional criteria should be used to distinguish AUL and various subtypes of AML (Ml to M7 and M6Er) and to differentiate MDS, MDS-ER, chronic myeloid leukemias, and leukemoid reaction. Bone marrow core biopsy and electron microscopy may be required to confirm the specific diagnosis. Immunophenotyping with lineage specific antibodies is in its infancy in veterinary medicine. Development of this technique is encouraged to establish an undisputed identity of blast cells. Validity of the proposed criteria needs to be substantiated in large prospective and retrospective studies. Similarly, clinical relevance of cytomorphologic, cytochemical, and immunophenotypic characterizations of AML in dogs and cats remains to be determined.

Journal Article↗

Feline myeloproliferative disease. Changing manifestations in the peripheral blood.

A 4 1/2-year-old cat had myeloproliferative disease characterized by severe anemia. Peripheral blood had a profusion of relatively normal-appearing nucleated erythroid cells in all stages of development and a few primitive cells with nucleoli. The term "erythremic myelosis" best described the appearance of the peripheral blood. Two weeks later, the peripheral blood picture was that of regenerative anemia. During the next 2 months the cat made an apparent clinical recovery without treatment, but relapsed 3 months after the initial examination. One-fourth of the cells in circulation at the time of relapse contained nucleoli and appeared identical to those described in reticuloendotheliosis in cats. This transition suggested the progressive development of a greater degree of immaturity of erythroid precusors with time. The use of the term "reticuloendotheliosis" in conjunction with feline myeloproliferative disease was considered and determined inappropriate.

Animals↗

[Subacute idiopathic autoimmunehemolytic anemia with prolonged aplastic phase and erythremic reaction (author's transl)].

A case of idiopathic autoimmune hemolytic anemia with subacute onset and peculiar course is reported. In the beginning of the disease there was a long lasting severe aplastic phase, during which numerous blood transfusions were required. The low antiglobuline test titre compared with the severe hemolytic activity and the ineffective erythropoesis suggested first of all the presence of pure red cell anemia. Later on blood regeneration with erythroblastosis started and resembled the picture of erythremic myelosis. The transient course of the disease with full normalisation of all blood values and continuous remission lasting now for more than one year is a peculiar variant of idiopathic autoimmune hemolytic anemia.

Aged↗

Aberrant megakaryocytopoiesis preceding radiation-induced leukemia in the dog.

Six of nine decedent beagles exposed continuously to 2.5 R*/22 hour day of whole-body 60Co gamma-radiation died with myeloproliferative diseases: three cases of myelogenous leukemia and one each of monocytic leukemia, erythroleukemia, and erythremic myelosis. The three dogs that died with myelogenous leukemia had micromegakaryocytes and megakaryoblasts in the peripheral blood during the preleukemic phase when myeloblasts were not observed in the peripheral blood or in increased numbers in the bone marrow. In this study we have examined the megakaryocytes during the preleukemic period by a combination of light, transmission, and scanning electron microscopy. Morphologic abnormalities seen by light microscopy included mononucleated and binucleated forms, many with cytoplasmic blebs. The small mononuclear forms in the bone marrow tended to form clusters. Ultrastructural features included a paucity of both specific alpha granules and dense granules. The micromegakaryocytes showed dysgenesis of the demarcation membrane system. This membrane system appeared disorganized with a few dilated round, oval, or rarely, elongated vesicles and showed no evidence of platelet formation. The cells also had a paucity of endoplasmic reticulum, few mitochrondria, and sparse glycogen accumulations. The scarcity of cytoplasmic organelles gave a pale immature appearance to the cytoplasm. By scanning electron microscopy, the sponge-like surface of large mature megakaryocytes from unirradiated marrow contrasted with the characteristically smooth, topographically featureless surfaces of the micromegakaryocytes from preleukemic dogs.

Animals↗

Familial acute myeloid leukemia and DiGuglielmo syndrome.

A family is described in which two sisters developed acute myeloblastic leukemia (AML FAB M2) and erythroleukemia (FAB M6) at ages 60 and 53, 15 years apart. Their father was diagnosed as having chronic erythremic myelosis (DiGuglielmo syndrome) 24 years earlier at age 56. Cytogenetic analysis of bone marrow blasts in the patient with AML M2 revealed a complex hyperdiploid karyotype with clonal abnormalities -3, -5, del(5)(q13q23), -8, +i(8)(q10), -11, add(11) (q23), add(12)(p13), -13, +3mar. No environmental risk factors could be identified. Hematologic and cytogenetic analysis of all living first degree relatives of the affected persons revealed hematological abnormalities, but normal constitutional karyotypes of peripheral blood lymphocytes and no induction of cytogenetic abnormalities in folate deficient medium.

Aged↗