Cases reported "Anemia, Myelophthisic"

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1/4. Clinical spectrum of myelophthisis in cancer patients.

    Myelophthisis is a form of bone marrow failure due to replacement of hematopoietic tissue by abnormal tissue, most commonly metastatic carcinomas. This results in extramedullary hematopoiesis, typically in the spleen leading to premature release of hematopoietic cells into the circulation. Peripheral blood findings may include nucleated red blood cells, tear drop forms, giant platelets, and immature leukocytes. This is called a leukoerythroblastic picture. The first case demonstrates acute myelophthisis as a presentation of pancreatic cancer. The second case is of extramedullary hematopoiesis as a manifestation of widely metastatic melanoma. The presence of a leukoerythroblastic peripheral blood picture should serve as a valuable clue about a possible underlying malignancy. This late presentation of advanced cancer may now be rarely seen because of early diagnosis and more effective therapies.
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2/4. Biochemical values, complement levels, and hemostatic data in septic leukoerythroblastosis.

    Recently, the association of granulocytic fragments on blood smear with leukoerythroblastosis in sepsis has been identified in nine patients. Granulocytic fragments were identified by both light and electron microscopy as well as cytochemistry. Leukoerythroblastosis is a poorly defined, uncommon syndrome with leukocytosis, left shift, and nucleated red blood cells (nRBCs) disproportionate to the degree of anemia, which may be associated with leukemia or neoplasia in the bone marrow, acute infection, hemolysis, myelofibrosis, or miscellaneous causes. Here a subgroup with high white blood cells (WBC) and acute infection was studied. The corrected WBC for nine patients was 40 x 10(9) per L with 33 nRBC per 100 WBC; serum C3 and C4 levels before and after the development of leukoerythroblastosis were 0.6 /- 2 g per L; 0.18 /- 0.04 g per L pre-leukoerythroblastosis and 0.7 /- 0.46 g per L; 0.30 /- 0.27 g per L post-leukoerythroblastosis, respectively, in four patients. The platelet count, prothrombin time (PT), and activated partial prothrombin time (aPTT) were 133 x 10(9) per L, 24.4 sec., and 53.5 sec., respectively, for nine patients. Multiphasic chemistries at the time of leukoerythroblastosis were measured in five patients; abnormal values included calcium of 2.0 /- 0.4 mmol per L, creatinine of 336 /- 130 mumol per L, total protein of 45 /- 17 g per L, albumin of 27 /- 11 g per L, total bilirubin of 421 /- 362 mumol per L, uric acid of 499 /- 264 mumol per L, triglycerides of 4.9 /- 3.7 mmol per L, and alkaline phosphatase of 3.5 /- 1.0 mu kat per L.(ABSTRACT TRUNCATED AT 250 WORDS)
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3/4. Granulocytic fragments in sepsis.

    We report here three patients with sepsis and one with acute pancreatitis and possible sepsis who developed granulocytic fragments on blood smears obtained prior to death. In case 1, these fragments were identified cytochemically. In case 3, granulocytic cytoplasmic projections and fragments were identified by electron microscopy of the buffy coat. All patients had leukerythroblastosis. The average corrected white blood count (WBC) was 46 X 10(9)/liter with 34 nucleated red blood cells (nRBC)/100 WBC. Patient 1 had thrombocytosis whereas patients 2, 3, and 4 were thrombocytopenic. Terminal complement levels were decreased in patients 3 and 4 as previously noted in sepsis (Sprung CL, Shultz DR, Marcial E, et al.: complement activation in septic shock patients. Crit Care Med 14:525, 1986). A general correlation between nRBC and granulocytic fragments/100 hpf (high power field) was observed in patients 3 and 4. Granulocytic fragments were not identified on the blood smears of several patients with leukemoid reactions without erythroblastosis. Although the precise etiology of these fragments is unclear, we believe their recognition is important because all patients died within 32 hours after granulocytic fragments were identified. Furthermore, these fragments can falsely elevate the platelet count. Although myeloid fragments have previously been noted in leukemia and lymphoma, this is the first report of their association with conditions unrelated to hematologic neoplasms. These fragments can easily be recognized by careful examination of the blood smear and represent a newly recognized aspect of the septic shock syndrome.
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4/4. Transient erythroblastopenia of childhood (TEC) presenting as leukoerythroblastic anemia.

    PURPOSE: The diagnosis of transient erythroblastopenia of childhood (TEC) is usually straightforward, with temporary cessation of red blood cell production resulting in normocytic normochromic anemia, reticulocytopenia, and bone marrow erythroblastopenia. We describe here a case of TEC presenting with features of leukoerythroblastic anemia. To our knowledge, this is the first such report for TEC. PATIENT AND methods: The case of a 1-year-old girl is described who had a leukoerythroblastic anemia. bone marrow examination and clinical course indicated that the anemia had a benign etiology-TEC. RESULTS: The patient presented with anemia, leukocytosis, and a left shift, with metamyelocytes, myelocytes, myeloblasts, and nucleated red cells in the circulation. There was no apparent viral etiology, and the bone marrow aspirate findings were consistent with a recovering marrow. After transfusion, the patient had an uneventful recovery from TEC. CONCLUSION: TEC can cause leukoerythroblastic anemia. TEC with this presentation is clinically similar to TEC without leukoerythroblastosis, but other causes of leukoerythroblastosis need to be excluded.
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