A tired old lady
Tim French, Terry Wardle in The Problem-Based Learning Workbook, 2022
Pancytopenia is a reduction in the peripheral circulation of red cells, white cells, and platelets. Generally pancytopenia is caused by decreased cell production in the marrow, or increased cell destruction by an enlarged spleen. When a reduction in all cell lines is observed, the main diseases that need to be excluded are: acute leukaemia (AML or ALL)myelodysplasia (MDS)myelomamarrow infiltration by lymphoma or solid tumoursaplastic anaemia (idiopathic, or secondary to drugs, infection, or radiation exposure)splenomegaly.
Unexplained Fever In Hematologic Disorders Section 1. Benign Hematologic Disorders
Benedict Isaac, Serge Kernbaum, Michael Burke in Unexplained Fever, 2019
A distinct form of severe anemia, usually normocytic or mildly macrocytic, is aplastic anemia,51 due to bone marrow failure. The peripheral blood usually shows anemia associated with leukopenia and thrombocytopenia (pancytopenia). The reticulocyte count is low. The bone marrow biopsy will reveal absent or reduced cellularity. The serum iron is elevated. The clinical manifestations vary with the severity of the pancytopenia. Bleeding and infection are the most common complications. Infection is usually associated with fever, but local signs of inflammation may be absent. The differential diagnosis is important because pancytopenia may occur in a variety of disorders, potentially evolving with fever, including lymphoma, pre-leukemia, metastatic carcinoma to the marrow, granulomatous diseases, parenchymal liver diseases with hypersplenism, and lipidoses. Two other diseases, usually febrile, SLE and tuberculosis due to atypical mycobacteria, may be associated with pan-yctopenia; some viral diseases, especially hepatitis and infectious mononucleosis, have also been implicated. The main causes of aplastic anemia are ionizing irradiation and chemical agents. The latter include chloramphenicol, phenylbutazone, hydantoin, sulfa drugs, phe-nothiazines, quinacrune, colchicine, sulfonylureas, gold compounds, benzene, insecticides, organic arsenicals, antineoplastic, and immunosuppressive drugs.
Miscellaneous Forms of Acquired Red Cell Aplasia and Erythropoietic Failure in Childhood
Stephen A. Feig, Melvin H. Freedman in Clinical Disorders and Experimental Models of Erythropoietic Failure, 2019
Although aplastic anemia is a well-reported complication of hepatitis, PRCA has been infrequently reported41,42 The myelosuppressive complications of hepatitis virus infection have almost invariably been associated with infection due to hepatitis C virus.43 Aplasia usually occurs after the symptoms of hepatitis, although it may occur coincidentally with hepatitis. Pancytopenia is usually severe, prolonged, and fatal. This is in contrast to the PRCA, which may be transient. Severe aplasia following hepatitis requires substantial supportive care, and bone marrow transplantation from a human leukocytic antigen (HLA)-matched sibling is the treatment of choice,44,45 but the predisposing liver damage may increase the risk of hepatic decompensation after transplantation. Alternate methods of management of posthepatitic aplasia, including supportive care, ATG, CyA, androgens, and corticosteroids, have been less effective. In considering the possibilities for transplantation, an early search for a matched sibling donor should be undertaken because long-term prognosis in the absence of transplantation is poor and sensitization to tissue antigens due to multiple transfusions may jeopardize the success of transplantation.
Deciphering the genotype and phenotype of hairy cell leukemia: clues for diagnosis and treatment
Published in Expert Review of Clinical Immunology, 2019
Margot C.E. Polderdijk, Michiel Heron, Saskia Kuipers, Ger T. Rijkers
Presenting symptoms are weakness, fatigue, fever, bleeding, splenomegaly, and pancytopenia, especially monocytopenia. However, the diagnosis is usually made based on the immunophenotype of the tumor cells, as shown in Table 1. Figure 2 shows the phenotypical expression of hairy cells as compared to normal B lymphocytes as determined by flow cytometry. Additional criteria that can function as indicators for the disease are cell morphology and cytogenetics (see below). Aside from the clinical symptoms, presence of hairy cells in the bone marrow or peripheral blood can be detected after taking a biopsy or blood sample of the patient. Historically, two types of HCL can be distinguished: classical and variant. The classical type is the most common, constituting about 90% of cases, while the variant type only appears in 10% of patients [10]. In addition, patients with the variant type are typically older, do not suffer from pancytopenia or monocytopenia, and more frequently show thrombocytopenia [11]. Moreover, the outcome of treatment is very different; chemotherapeutic agents that are responsible for the high cure rates seen in classical HCL are less effective in HCL variant [12]. Because of all these differences, HCL variant is now considered a separate disease rather than a subtype of HCL [1].
Bilateral periorbital leukemia cutis presenting as suspected cellulitis
Published in Orbit, 2022
Lalita Gupta, Melissa A. Levoska, Timmie Sharma, Kord Honda, Mark A. Prendes
Upon evaluation after transfer, the patient endorsed bilateral periorbital swelling, erythema, and tenderness and denied vision changes, diplopia, and pain with extraocular movements. Visual acuity with correction was at her baseline and measured to be 20/70 for the right eye due to a longstanding failed corneal graft and 20/40 for the left eye. There was normal ocular motility and no relative afferent pupillary defect. Examination showed edematous and erythematous nontender indurated plaques involving bilateral upper and lower eyelids and nasal bridge (Figure 1A). No vesicles were observed. Anterior segment examination was significant for corneal edema of the right eye in the setting of prior corneal graft failure and mild bilateral chemosis. The remainder of the eye examination was unremarkable. Further examination of the skin revealed other cutaneous lesions including a pink, firm papule on the right upper eyebrow and light pink nodules on the left anterior shoulder, left jawline, upper back and central abdomen. The patient was afebrile. Laboratory results were significant for pancytopenia.
Analysis of Mean Corpuscular Volume and Red Cell Distribution Width in Patients with Aplastic Anemia
Published in Hemoglobin, 2023
Lingling Liu, Qiuhao Fu, Danfeng Zhang, Dandan Chen, Fang Wang, Rong Guo, Xinsheng Xie, Zhongxing Jiang, Jifeng Yu, Yingmei Li
The inclusion criteria were as follows: (1) age ≥14 years, (2) presence of cytopenia as previously defined, (3) laboratory tests were performed, including complete blood count, bone marrow aspirate and biopsy, bone marrow karyotype analysis and fluorescence in situ hybridization, peripheral blood smear, serum iron, ferritin, serum Vitamin B12 and folate levels, lymphocyte subsets, flow cytometry for GPI-anchored proteins to detect paroxysmal nocturnal hemoglobinuria (PNH) clone, anti-nuclear antibody and anti-double stranded DNA, liver and kidney function tests, thyroid function tests, viral detection (hepatitis A/B/C, EBV, CMV, HIV and Parvovirus B19), abdominal ultrasound and echocardiogram. If the diagnosis cannot be determined, further pertinent examinations will be conducted, including next-generation sequencing and single-nucleotide polymorphism array karyotyping. The pancytopenia diagnosis was defined and classified in 731 patients. A total of 256 subjects were diagnosed with AA, including 180 with new-onset AA and 76 with post-treatment AA (as defined treatment more than 3 months). We analyzed the hemograms of 180 newly diagnosed AA patients and 166 MDS patients as controls.
Related Knowledge Centers
- Antibiotic
- Bone Marrow Suppression
- Chloramphenicol
- White Blood Cell
- Platelet
- Lymphocyte
- Red Blood Cell
- Monocyte
- Infectious Mononucleosis
- Complete Blood Count