Materials and Methods: A prospective study of 12 months' duration was carried out, which included patients of all age groups. Of the 60 patients presenting with pancytopenia, bone marrow aspiration and biopsy were done on 30 cases, after taking an informed consent. Patients who had received previous blood transfusion and were on chemo- and radiotherapy were excluded.
A detailed clinical examination of all cases was carried out. This was followed by acute myeloid leukemia Conclusion: The varied causes of pancytopenia can be attributed to the geographic area, genetic differences, stringency of diagnostic criteria, and differences in the methodology used.
- Fever, Hepatosplenomegaly, and Pancytopenia in a 5-Month-Old Infant.
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There are varying trends in its clinical pattern, treatment modalities, and outcomes. The severity of pancytopenia and the underlying pathology determines the management and prognosis.
Thus, identification of the correct cause will help in implementing the appropriate therapy. Users Online: Login. Pancytopenia - a clinico haematological study of cases. Indian J Pathol Microbiol ; Chandra K, Kumar P. Morphological spectrum of bone marrow in pancytopenia - a retrospective study in a tertiary care centre.
Pancytopenia: A Clinico Hematological Study
J Evol Med Dent Sci ; Bone marrow examination in cases of pancytopenia. J Indian Acad Clin Med ; Keisu M, Ost A. Diagnoses in patients with severe pancytopenia suspected of having aplastic anemia.
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- British Library EThOS: The pathogenesis of Bovine Neonatal Pancytopenia.
Eur J Haematol ; Pancytopenia: A clinico hematological study. The presence of pancytopenia or other focal hematologic abnormalities, such as leukopenia, thrombocytopenia, or anemia, makes an infiltrative process even more likely because the bone marrow can be similarly infiltrated and impeded from ongoing hematopoietic activity. Fever is often reported as a symptom in the initial presentation of such patients. Personally, however, I have often found these markers to be unreliable, and, subsequently, I agree that evaluation of the bone marrow is usually indicated, as was the case here.
Incidentally, bone marrow aspiration and biopsy are often underappreciated as diagnostic tests for nonhematologic or nononcologic disorders. Many metabolic and infectious diseases can be identified from stains or cultures of bone marrow material. In addition, although sometimes an uncomfortable procedure, bone marrow sampling is far safer and simpler than is liver biopsy and often more timely than awaiting blood tests, such as serologic titers. In this case, the bone marrow biopsy specimen did not show signs of a hematologic malignancy.
Although initially reassuring, the peripheral blood smears and bone marrow studies do not definitively rule out malignancy. However, I must now broaden my differential diagnosis and consider other etiologies. A somewhat reassuring caveat is that if this infant does have cancer, delaying the initiation of oncologic therapy for a short period of time while additional studies are performed will not likely affect the overall prognosis.
Furthermore, as mentioned earlier, I still am compelled to try and identify any simultaneous infectious disorders that might complicate initiation of therapy, for that underlying illness anyway. Initial tests for oncologic disease failed to yield a definitive diagnosis, so I next consider an infectious process. Because both the liver and spleen are integral parts of the reticuloendothelial system, the most likely cause of organomegaly in children is an inflammatory response to a systemic infection.
Hepatosplenomegaly is commonly associated with many systemic viral infections, including acute Epstein-Barr virus or cytomegalovirus infections, viral hepatitis hepatitis A, B, C, etc and HIV. Although lymphocytosis is often seen with acute viral illnesses, many viral illnesses can cause some transient suppression of bone marrow activity, particularly with transient leukopenias. Statistically, viral infections are the most likely cause of infections in children. Bacterial infections can also cause hepatosplenomegaly, both by activation of the reticuloendothelial system and via phagocytosis of organisms and macrophage engorgement.
Both processes may be involved to varying degrees by entities such as septicemia, tuberculosis, endocarditis, cholangitis, bartonellosis, and brucellosis. Bacterial sepsis can present with leukopenia or leukocytosis, although I find that leukopenia is both more common and a graver prognostic sign in infants than in older children. Less common infectious causes of hepatosplenomegaly in North America are parasitic eg, toxoplasmosis or malaria and fungal eg, histoplasmosis or disseminated candidiasis infections, which can present with acute, chronic, or subacute symptoms.
Evaluation for an infectious disease should focus on a detailed history of the tempo of developing symptoms to determine the acuity of the illness. Potential exposures should be carefully examined, including contacts with persons who may have tuberculosis, viral hepatitis, or mononucleosis. Other pertinent exposures may include cats and raw meat ingestion, which can result in toxoplasmosis or bartonellosis cat scratch disease. A history of blood transfusions or intravenous drug use in the mother raises the possibilities of HIV or hepatitis B or C.
Travel or residence in an area with endemic diseases, such as histoplasmosis or malaria, should be discussed.
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In this case, the patient has a unique risk factor that may be overlooked by physicians who do not practice in the midwestern United States. The infant lives in Indiana in a very old house that has recently undergone renovations, which puts her at high risk for exposure to histoplasmosis. Serologic testing for specific organisms may be indicated depending on the exposure history and clinical findings. Monospot examinations for Epstein-Barr virus testing for heterophile antibodies may be unreliable in very young children, and, therefore, specific Epstein-Barr virus and cytomegalovirus serologic testing may be more accurate and useful.
IgM and IgG antibody testing is available in most laboratories for both viruses. Serum transaminase levels and fractionated bilirubin levels assess the degree of hepatobiliary disease. Markedly elevated serum transaminase levels indicate the need for acute hepatitis serologic testing, including hepatitis A IgM, hepatitis B surface antigen, hepatitis B core IgM, and hepatitis C antibody, or RNA polymerase chain reaction. Placement of intradermal purified protein derivative for tuberculosis screening and intradermal controls to assess for anergy should be considered.
Serial large volume blood cultures are critical in detecting low-grade or intermittent bacteremia that can be seen with endocarditis. Bone marrow aspiration for hematopathologic examination may be necessary, and additional bacterial cultures and special stains for acid-fast bacilli mycobacterial infections and fungi should be included.
March 2008: A 14 y.o. with pancytopenia and massive splenomegaly
Specific testing for histoplasmosis will be discussed later in the article. If I were to ignore the fact that this patient had a prolonged history of fever and only focus on the hepatosplenomegaly with or without the hematologic abnormalities, I would have to address a broader differential diagnosis. Clearly, hepatosplenomegaly can occur as a result of noninfectious inflammatory conditions.
These include autoimmune disorders such as sarcoidosis and systemic lupus erythematosus, although most of these entities are apparent in a patient's history through a careful review of systemic symptoms and have additional findings on physical examination. Hepatosplenomegaly can also result from vascular congestion. Suprahepatic or posthepatic venous outflow obstruction may lead to backup into the liver and spleen. Examples include congestive heart failure, pericardial disease, and hepatic vein obstruction Budd-Chiari syndrome.
Intrahepatic obstructive diseases that can similarly cause hepatomegaly with biliary stasis and secondary congestive splenomegaly include cirrhosis, hemangiomas, focal nodular hyperplasias, and congenital fibrosis.
Also, because the liver is responsible for biosynthesis and storage of nutritional materials, metabolic disorders can cause hepatomegaly. The liver stores carbohydrates as glycogen and buffers serum glucose levels through gluconeogenesis. Circulatory proteins, including albumin, complement components, and secretory and transport proteins as well as lipoproteins and lipids, are also synthesized and stored. Because of the metabolic functions of the liver, inborn errors of metabolism and other congenital enzymatic deficiencies can result in increased accumulation of both intracellular and extracellular metabolic by-products.
Typically, such derangements present in the first years of life. Examples include storage disorders of lipids, such as Gaucher disease and Niemann-Pick disease, and a variety of glycogen storage diseases, including Hurler disease. These are often associated with other physical findings, such as abnormal craniofacial features. Any of these disorders can gradually result in displacement or replacement of the normal function of the bone marrow compartment. Other disorders resulting primarily in hepatomegaly include Wilson disease, hypervitaminosis A, and hemochromatosis.
Symptoms of all such disorders generally present subacutely and are rarely associated with fever. Returning to the details of this case, the bone marrow biopsy specimen revealed several histiocytes containing intracellular yeastlike forms Figure 2 and Figure 3. For reasons mentioned above, this infant is at high risk for exposure to Histoplasma capsulatum and, as such, this organism is the most likely culprit.
March A 14 y.o. with pancytopenia and massive splenomegaly – California Tumor Tissue Registry
Disseminated Histoplasmosis of Infancy Jason M. Kane, MD : This case demonstrates the classic presentation of progressive disseminated histoplasmosis of infancy. Histoplasmosis, caused by the thermally dimorphic fungus H capsulatum , is the most common primary systemic mycosis in the United States. The organism is endemic in certain areas of Latin America and the United States, where most cases originate in the Ohio, Missouri, and Mississippi River valleys. Bird and bat excrement enhance the growth of the organism, and aerosolization of the microconidia occurs when the contaminated soil is disturbed, such as with areas of new construction or with renovation of old buildings or houses.
The severity of illness after inhalation of H capsulatum varies depending on the intensity of exposure and the immunity of the host. K Takatsuki K Takatsuki. Blood 85 5 : This content is only available as a PDF. Volume 85, Issue 5. Previous Article Next Article. View Metrics. Cited By Google Scholar. Email alerts Article Activity Alert. First Edition Alert. Latest Issue Alert.