1/8. The psuedo-Chediak-Higashi anomaly: an unusual staining pattern in an Indian child with acute myeloid leukemia.The authors describe the psuedo-Chediak-Higashi anomaly in a 12-year-old boy with acute myeloid leukemia (AML-M2). There were large purple granules in the blasts, promyelocytes, and myelocytes. Instead of the previously described patterns, the authors observed a unique rim pattern staining of the granules in both the May-Grunwald-Giemsa and the myeloperoxidase stains. Moreover, many of the granules had central vacuoles with strong myeloperoxidase positivity at the periphery. The bone marrow had a much higher positivity for these mega-granules as compared to the peripheral blood. On remission, these granules were no longer seen. To the best of the authors' knowledge, this pattern of staining has not been previously reported in the literature.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
2/8. Acute myeloid leukemia with pseudo-Chediak-Higashi anomaly exhibits a specific immunophenotype with CD2 expression.Acute myeloid leukemia (AML) with pseudo-Chediak-Higashi (PCH) anomaly is a rare morphologic entity. We characterized 5 cases by multiparameter flow cytometry and found that in all cases, the blasts aberrantly expressed CD2, a pan-T cell-associated marker, in addition to their myeloid-associated markers. In contrast, CD2 was expressed in only 25 (17.9%) of 140 cases of newly diagnosed AML without PCH anomaly. CD2 expression correlated strongly with AML with PCH anomaly (P < .01), suggesting a link between a specific immunophenotypic marker, CD2, and AML with PCH anomaly.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
3/8. Epstein-Barr virus infection in chediak-higashi syndrome mimicking acute lymphocytic leukemia.We have had the opportunity to study a case of chediak-higashi syndrome (CHS) in the accelerated phase that was associated with Epstein-Barr virus (EBV) infection. The clinical course of a 12-year-old boy was characterized by fever, lymphadenopathy, hepatosplenomegaly, and pancytopenia. However, in the terminal stage, the appearance of an atypical lymphoblastic leukocytosis was morphologically indistinguishable from acute lymphocytic leukemia, accompanied by benign histiocytosis with hemophagocytosis. autopsy examination revealed an atypical lymphoid infiltration favoring EBV infection as the primary diagnosis. This case underscores the fatal consequences of EBV infection in CHS.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
4/8. Pseudo-Chediak-Higashi anomaly in a case of acute myeloid leukemia: electron microscopic studies.The formation and fine structure of giant granules in neutrophil promyelocytes of a patient with a variant of acute myelogenous leukemia were investigated by electron microscopy. The patient presented with large lymph nodes and disseminated intravascular coagulation (DIC). By light microscopy, numerous giant granules, resembling those of chediak-higashi syndrome (CHS), were present, but Auer bodies could not be found. By electron microscopy, these giant granules were seen to be formed by fusion of azurophilic granules, as in CHS; however, they were different from the large granules of CHS, since they contained numerous microcrystalline structures like those of Auer bodies. However, the crystalline cores of these granules exhibited a periodicity different from that of Auer bodies of acute promyelocytic leukemia. This clinical and hematologic syndrome (giant granules, enlarged lymph nodes, and DIC may represent a variant of acute promyelocytic leukemia.- - - - - - - - - - ranking = 1.4keywords = leukemia (Clic here for more details about this article) |
5/8. Pseudo-Chediak-Higashi anomaly.Pseudo-Chediak-Higashi (PCH) anomaly was seen in a patient with a myelodysplastic syndrome evolving from pancytopenia with normocellular marrow to a myeloblastic leukemia over a period of 72 months. This case is reported.- - - - - - - - - - ranking = 0.2keywords = leukemia (Clic here for more details about this article) |
6/8. A cytochemical and ultrastructural study of acute myelomonocytic leukemia exhibiting the pseudo-Chediak-Higashi anomaly of leukemia and "splinter-type" Auer rods.An unusual case of acute myelomonocytic leukemia (AMML) in an 81-year-old man is described. Ultrastructural examination revealed the presence of distinct Auer bodies, which commonly are found in acute promyelocytic leukemia (APL). The Auer bodies had a splinterlike appearance with a distinct tubular substructure. The morphologic appearance of the leukemic cells coupled with the results of cytochemical staining reactions (alpha-naphthyl AS-D chloroacetate esterase, alpha-naphthyl butyrate esterase, sudan black B, and periodic acid-schiff reaction) identified this leukemia as myelomonocytic in origin. APL-like Auer rods in AMML now can be included in the spectrum of morphologic forms commonly seen in myelogenous leukemia and lends support to the hypothesis of an aberrant stem cell. In addition to the APL-like Auer rods, the leukemic blasts contained giant pink-staining granules on Wright's stain, some of which had a tubular substructure similar to the Auer rods under the electron microscope. The presence of these megagranules is a morphologic aberration termed "the pseudo-Chediak-Higashi anomaly of acute leukemia," and now has been described for the first time in AMML at the ultrastructural level.- - - - - - - - - - ranking = 2.6keywords = leukemia (Clic here for more details about this article) |
7/8. Electron microscopic and peroxidase cytochemical analysis of pink pseudo-Chediak-Higashi granules in acute myelogenous leukemia.Giant round pink inclusions (congruent to 2 micrometers) were seen in neutrophilic myeloblasts, promyelocytes, and myelocytes from three patients with acute myelogenous leukemia. On preliminary examination of the bone marrow smears, these inclusions looked like ingested red blood cells in that they were pink and not azurophilic. The bone marrow specimens were processed for the electron microscopic demonstration of peroxidase with 3,3'-diaminobenzidine and H2O2 at pH 7.6. In all three cases, the inclusions were determined to be large peroxidase-positive granules since they were limited by a single unit membrane and, unlike endocytized red blood cells, were not contained within phagocytic vasuoles. The granules were homogeneously dense for peroxidase and showed no obvious crystalline structure when examined stained or unstained on grid. We believe that they correspond to the giant pink round granules Van Slyck and Rebuck observed in immature leukemic granulocytes in 1974 and termed the pseudo-Chediak-Higashi anomaly. Like the giant purple granules seen in leukemia with this anomaly, these granules also appear to be an abnormal variant of peroxidase-positive azurophil (primary) granules. Their lack of azurophilia is due to the absence of sulfated glycoaminoglycans.- - - - - - - - - - ranking = 1.2keywords = leukemia (Clic here for more details about this article) |
8/8. Acute promyelocytic leukemia with the pseudo-Chediak-Higashi anomaly and molecular documentation of t(15;17) chromosomal translocation.Two morphologic variants of acute promyelocytic leukemia (AML M3) are recognized--the hypergranular form, in which Auer rods are usually numerous; and the microgranular form. The case of AML M3 reported here lacked numerous Auer rods but was hypergranular and demonstrated prominent giant Chediak-Higashi-like granules in the leukemic cells. Although routine karyotypic studies were inconclusive, at t(15;17) translocation was documented using Southern blot genotypic analysis and probes for the retinoic acid receptor-alpha and pml genes. This is the first case of this unusual morphologic anomaly in which the granules are described in routine histologic sections and the first with evidence of a t(15;17) chromosomal translocation. It also illustrates the importance of Southern blot analysis in suspected cases of AML M3 with "negative" karyotypic studies.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |