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Purpose: The purpose of this study was to determine the capability of proton density with fat fraction (PD-FFQ) imaging to help assess hematopoietic ability and diagnose aplastic anemia in adults., Methods: Between January 2021 and March 2023, patients diagnosed with aplastic anemia (AA: n = 14) or myelodysplastic syndrome (MDS: n = 14) were examined by whole-body PD-FFQ imaging, and 14 of 126 age and gender matched patients who had undergone the same PD-FFQ imaging were selected as control group. All proton density fat fraction (PDFF) index evaluations were then performed by using regions of interest (ROIs). Pearson's correlation was used to determine the relationship between blood test results and each quantitative index, and ROC-based positive test and discrimination analyses to compare capability to differentiate the AA from the non-AA group. Finally, sensitivity, specificity and accuracy of all quantitative indexes were compared by means of McNemar's test., Results: Mean PDFF, standard deviation (SD) and percentage of coefficient of variation (%CV) for vertebrae showed significant correlation with blood test results (-0.52 ≤ r ≤ -0.34, p < 0.05). Specificity (SP) and accuracy (AC) of %CV of PDFF in vertebrae were significantly higher than those of mean PDFF in vertebrae and the posterior superior iliac spine (SP: p = 0.0002, AC: p = 0.0001) and SD of PDFF in vertebrae (SP: p = 0.008, AC: p = 0.008). Moreover, AC of SD of PDFF in vertebrae was significantly higher than that of mean PDFF in vertebrae and the posterior superior iliac spine (p = 0.03)., Conclusion: Whole-body PD-FFQ imaging is useful for hematopoietic ability assessment and diagnosis of aplastic anemia in adults., Competing Interests: Declaration of competing interest None., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Abstract: We present the case of a 23-year-old woman with juvenile onset systemic lupus erythematous on a background of thrombotic thrombocytopenic purpura, who was referred for 18 F-FDG PET CT scan due to pyrexia of unknown origin with raised inflammatory markers, severe thrombocytopenia, and anemia. An interesting pattern of predominantly photopenic hypometabolic bone marrow activity was demonstrated on 18 F-FDG PET CT., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Anemia is an important clinical symptom for aplastic anemia (AA) patients who are suffered with peripheral pancytopenia., Objective: To evaluate the accuracy of diagnosing anemia with non-invasive chest computed tomography (CT) for AA patients., Methods: The CT attenuation of left ventricular (LV) cavity and interventricular septum (IVS) on unenhanced thoracic CT images of AA patients are retrospectively analyzed, including 84 AA patients in pre-transplant and 1-month (n = 82), 2-month (n = 72), 3-month (n = 75), 6-month (n = 74) and 12-month (n = 70) followed patients in post-transplant. The difference (IVS-LV) and ratio (LV/IVS) of the CT attenuation between LV cavity and interventricular septum are calculated. Serum hemoglobin is estimated within 24 hours of CT imaging. The CT attenuations of IVS-LV and LV/IVS are correlated with hemoglobin, and their variation tendency is analyzed during the treatment of a-HSCT. A receiver operating characteristic (ROC) curve analysis is then performed for the diagnosis of anemia., Results: The CT attenuations of IVS-LV and LV/IVS well correlate with hemoglobin (r = -0.618 and 0.628, respectively, P <  0.001). The variation tendency of IVS-LV and LV/IVS is similar to that of hemoglobin with opposite directions during one-year follow-up of a-HSCT. When a threshold of CT attenuation of IVS-LV and LV/IVS is set at 11.5HU and 0.77, respectively, both the sensitivity and specificity in diagnosing anemia are good (74.7% and 73.8% in CT attenuation of IVS-LV; 77.4% and 70.4% in LV/LVS, respectively)., Conclusions: Both CT attenuation of LV/IVS and IVS-LV had similar accuracy in diagnosing anemia for AA patients. The non-invasive chest CT can offer a new possibility to complementarily evaluate anemia for AA patients in the diagnostic radiology reports.

Purpose: Peripheral cytopenias are typical of blood test abnormalities associated with a variety of conditions, including aplastic anemia (AA) and myelodysplastic syndromes (MDSs). We prospectively investigated the feasibility of quantitative analysis of whole-body bone marrow activity using PET with 3'-deoxy-3'- 18 F-fluorothymidine ( 18 F-FLT) in AA and MDS., Patients and Methods: Sixty-eight patients with cytopenia underwent 18 F-FLT PET/MRI scan, with simultaneous bone marrow aspiration and biopsy for hematopoiesis evaluation. SUVs were measured in the vertebrae (Th3, 6, and 9 and L3), bilateral iliac crests, and extremities. SUV and bone marrow pathology were compared between AA and MDS and analyzed in relation to severity of AA and prognosis of MDS., Results: Of the 68 patients with cytopenia, 12 were diagnosed with AA, 27 with MDS, 12 with bone marrow neoplasia, 2 with myelofibrosis, and 15 with other conditions. Iliac 18 F-FLT SUVs were significantly correlated with bone marrow cell numbers and cell density ( r = 0.47, P < 0.001 and ρ = 0.65, P < 0.001, respectively). There was a significant positive correlation between iliac and vertebral SUVs in AA and MDS ( r = 0.65, P < 0.05 and r = 0.70, P < 0.001, respectively), and the slope of the regression line was significantly steeper in AA than in MDS ( P < 0.05). In AA patients, vertebral 18 F-FLT SUVs significantly decreased with disease progression, and in MDS patients, higher whole-body 18 F-FLT uptake was associated with shorter overall survival (hazards ratio, 3.18; 95% confidence interval, 1.07-9.47; P = 0.037)., Conclusions: Quantitative whole-body bone marrow imaging using 18 F-FLT PET helps distinguish AA from MDS and assess the severity of AA and prognosis of MDS., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2022 The Author(s). Published by Wolters Kluwer Health, Inc.)

Background: Bone marrow of patients with aplastic anemia (AA) is different from that of patients with myelodysplastic syndrome (MDS) and is difficult to identify by blood examination. IDEAL-IQ (iterative decomposition of water and fat with echo asymmetry and least-squares estimation) imaging might be able to quantify fat fraction (FF) and iron content in bone tissues., Purpose: To determine if IDEAL-IQ measurements of bone marrow FF and iron content can distinguish between patients with AA and MDS., Study Type: Retrospective., Population: Fifty-seven patients with AA, 21 patients with MDS, and 24 healthy controls., Field Strength/sequence: 3.0 T, IDEAL-IQ sequence., Assessment: Three independent observers evaluated the IDEAL-IQ images and measured FF and R2* in the left posterior superior iliac spine., Statistical Tests: Kruskal-Wallis test, linear correlations, and Bland-Altman analysis were used. A P-value of <0.05 was considered statistically significant., Results: The FF in patients with AA (79.46% ± 15.00%) was significantly higher than that in patients with MDS (42.78% ± 30.09%) and control subjects (65.50% ± 14.73%). However, there was no significant difference in FF between control subjects and patients with MDS (P = 0.439). The R2* value of AA, MDS, and controls was 145.38 ± 53.33, (171.13 ± 100.89, and 135.99 ± 32.41/second, respectively, with no significant difference between the three groups (P = 0.553)., Data Conclusion: Quantitative IDEAL-IQ magnetic resonance imaging may facilitate the diagnosis of AA and distinguish it from MDS., Level of Evidence: 3 TECHNICAL EFFICACY STAGE: 2., (© 2021 The Authors. Journal of Magnetic Resonance Imaging published by Wiley Periodicals LLC on behalf of International Society for Magnetic Resonance in Medicine.)

Hepatitis-associated aplastic anemia is a well-recognized clinical syndrome in which marrow failure follows the development of hepatitis. Although aplastic anemia is intimately related to paroxysmal nocturnal hemoglobinuria, until now, no cases of PNH-associated hepatitis have been described. We report a case of recurrent acute hepatitis preceding the clinical onset of PNH. Treatment of PNH with the complement inhibitor eculizumab (Soliris ® ) prevented both recurrences of episodes of intravascular hemolysis and liver enzyme alteration. This is the first known published case of PNH-associated hepatitis.

Background: The purpose of this study is to investigate the performance of dual-layer detector spectral CT for iron deposition compared to magnetic resonance imaging (MRI) T2* imaging., Methods: Thirty-one patients with a clinical history of myelodysplastic syndromes and aplastic anemia underwent liver and cardiac T2*-weighted unenhanced MRI on a three-tesla MRI scanner, and underwent unenhanced CT scan laterally on a 128-row spectral detector CT. R2* values of the liver, septal muscle, and paraspinal muscle were calculated. Attenuation differences (ΔH) in the liver and myocardium were calculated between the lower (50 keV) and higher (120 keV) energy levels., Results: The liver and cardiac T2* values were 9.54 ± 5.63 ms and 21.41 ± 2.44 ms, respectively. The liver-to-muscle and myocardium-to-muscle T2* value ratios were 0.37 ± 0.23 and 0.79 ± 0.19, respectively. The liver and cardiac ΔH were - 1.13 ± 4.24 HU and 2.22 ± 4.41 HU, respectively. There was a strong linear correlation between the liver R2* and ΔH (r = - 0.832, P < 0.001), but weak correlation existed between the cardiac R2* and ΔH (P = 0.041)., Conclusions: Dual-layer detector spectral unenhanced CT seemed to be equally valuable to MRI T2* imaging for evaluating liver iron overload.

The diagnosis and monitoring of aplastic anemia (AA) rely heavily on a complete blood count (CBC), and multiple-site bone marrow (BM) aspirations and biopsies. However, these approaches have certain limitations. We aimed to assess high-resolution magnetic resonance imaging (MRI) as a complementary approach for evaluating BM hypoplasia and monitoring treatment response in adults with AA in the current era.Twelve newly diagnosed AA patients and 12 sex- and age-matched healthy controls were enrolled in this study from January 2017 to August 2018. A bilateral iliac 3.0T MRI was used to collect data for each subject, and the signal intensity on the T1-weighted images (T1WIs) were expressed as a contrast-to-noise ratio (CNR). The MRI, CBC, and BM biopsy data were analyzed and compared.A qualitative analysis identified a significant difference in MRI signal characteristics between the AA group and the healthy control group. The clinical classifications of very severe aplastic anemia (VSAA) and severe aplastic anemia (SAA) corresponded to pattern I and pattern II on the MR images, respectively. However, this imaging classification did not correlate with the biopsy-based BM cellularity measure. A quantitative analysis showed a significantly higher signal intensity in AA patients than in controls. A within-group comparison revealed that more severe types of AA, based on the clinical classification, corresponded to stronger signals. Notably, MRI could detect treatment response earlier than CBC, regardless of whether there were improvements in hematopoiesis.MRI can be used to predict the therapeutic effects in patients with AA and is an important complementary tool for evaluating and monitoring BM hypoplasia.

Competing Interests: Competing interests: None declared.

Several studies suggest that cerebral microbleeds (CMB) seen on gradient echo magnetic resonance imaging (GE-MRI) of brain increase the future risk of intracranial hemorrhage (ICH). We investigated 26 newly diagnosed patients of aplastic anemia (AA) with GE-MRI of brain who were otherwise neurologically asymptomatic. These patients were then further followed up for a period of 6 months for development of overt ICH. The median age of patients was 20.5 years (range 12-40 years). The median values of complete blood counts at the time of diagnosis were hemoglobin 5.7 gm/dl (range 2.5-9.2 gm/dl), white cell count 2,320/μl (800-7,000/μl), and platelet count 11,500/μl (7000-45,000/μl). Three patients were detected to have CMB while two patients had asymptomatic cerebral macrobleeds (>1 cm). During the follow-up period of 6 months, two patients developed spontaneous ICH. None of the two patients had CMB at diagnosis. In conclusion, asymptomatic CMB were found in 11.5 % of patients with AA. The overall prevalence of asymptomatic ICH (CMB & macrobleeds) was 19.2 %. Further studies with larger number of patients are required to clearly delineate the association.

Epstein-Barr virus-associated post-transplant lymphoproliferative disease (EBV-PTLD) is a life-threatening complication following allogeneic hematopoietic stem cell transplantation (HSCT). Monitoring of EBV DNA in high-risk patients with subsequent pre-emptive rituximab treatment is highly effective, and can prevent EBV-associated disease following HSCT. Here, we report a 10-year-old girl with aplastic anemia who developed CD20 negative EBV-PTLD after unrelated bone marrow transplantation that was refractory to rituximab treatment. Similar to other types of lymphoma, the absence of CD20 antigen is likely to be characteristic of rituximab-refractory EBV-PTLD.

Rationale: A discordant relationship between bone marrow cellularity and peripheral blood findings is regularly noticed in patients with aplastic anemia (AA). Therefore, the feasibility of 3-F-18 fluoro-3-deoxy-L-thymidine (F-18 FLT PET was tested as a noninvasive tool to visualize the total distribution of the hematopoietic bone marrow compartment in AA at presentation or after treatment., Methods: In vivo scanning was performed with F-18 FLT PET in AA patients (n = 17), including patients upfront (n = 11) and following treatment (n = 6), in addition to peripheral blood cell counts and a bone marrow biopsy., Results: A striking abnormal F-18 FLT scan was observed in all patients upfront treatment, in particular a reduced uptake of the pelvis was shown, the area that is biopsied for the bone marrow biopsy. Following treatment, the number of solitary lesions with increased proliferative activity outside the pelvis was noticed in patients with partial response, whereas patients with a complete remission showed a homogenous uptake throughout the skeleton., Conclusion: This pilot study demonstrates that F-18 FLT scan provides a highly distinctive overview of the bone marrow compartment in AA that might be helpful for making a proper diagnosis and monitoring treatment response of AA patients.

We investigated the hematopoietic status of aplastic anemia with FDG-PET before and after immunosuppressive therapy. FDG-PET showed a patchy uptake pattern before treatment, indicating residual compensatory hypercellular marrow. Three years after successful treatment with ATG plus CsA, the heterogeneity of bone marrow uptake persisted, suggesting that expanded reconstitution of hematopoiesis may require a long time even after the achievement of hematological remission.

Aplastic anaemia (AA) in connection with liver damage is characterized by severe course of the disease, significant shift of the myelogram, considerable changes of blood picture, high lethality, taking in this pathological process various organs and digestion systems. Results of ultrasonic and biochemical researches show presence of deep changes in the liver, gall bladder and pancreas. The disbalance of lipid peroxidation and antioxidative protection system have been observed in these patients. It is revealed by decrease in the activity of enzymes of superoxide dismutase, glutathione peroxidase and catalase, in increase in capacity of intermediate and end products of POL. It is especially expressed in combination of AA with liver damage. That's why it is necessary to provide a constant control of these organs.

Introduction: Nodular regenerative hyperplasia (NRH) is a rare condition is associated to immune, neoplastic, vascular disorders, and certain drugs and toxins. Portal hypertension is the main complication., Aim: To report the case of a woman with NRH and aplastic anemia, association not previously described., Case: A 31- year-old hispanic female presented with pancitopenia and cholestasis. Bone marrow biopsy: aplastic anemia. Abdominal ultrasound revealed multiple liver nodules, without hepato-splenomegaly. No abnormalities were noted in hepatic vessels. Antimitochondrial (AMAs) were positive. Coagulation tests were normal. Liver biopsy: hepatocite nodules with central atrophy and sinusoidal dilatation in the absence of significant fibrosis. No arteritis, thrombosis or bile duct damage was found., Discussion: Pathogenesis of NRH remains unclear. The most accepted theory was developed by Wanless 24 years ago, and reinforced in 1997. It states that alterations in hepatic blood flow (portal obliterative venopathy) originate a specific response, initially consisting of apoptosis secondary to acute ischemia, with ensuing parenchymal atrophy and surrounding areas of liver regeneration and chronic ischemia. The common pathologic mechanism of all the diseases associated with NRH involves liver blood-flow abnormalities secondary to microthromboses, vascular congestion, or vasculitis. In the current case we were unable to document evidence of liver flow abnormalities. Noteworthy, there are previous descriptions of the as- sociation of NRH with primary biliary cirrhosis, and this patient was AMA positive, but without bile duct damage in liver biopsy., Conclusions: We cannot sustain Wanless' theory, and this is the first description of the association of NRH with aplastic anemia.

The incidence and clinical and magnetic resonance imaging features of osteonecrosis of the hip were evaluated in patients with aplastic anemia. Two hundred and forty-one patients with aplastic anemia were examined using MR imaging of bone marrow during the five years from 1994 to 1998. Osteonecrosis of the hip was observed on MR imaging in nineteen (15 males and 4 females, mean age 35 yr) of the 241 patients. It was present in both hips in 14 patients, and there were five cases with unilateral occurrence, with a total of 33 involved hips. All except for five hips with associated bone marrow edema revealed increased fatty marrow conversion in the proximal femoral metaphysis. In nine patients, osteonecrosis was detected without any pain. Five patients already had osteonecrosis before any medication was administered. Twelve patients received antilymphocyte globulin, and seven patients received a low dose of steroids before the MR diagnosis of osteonecrosis. Osteonecrosis of the hip frequently develops in patients with aplastic anemia (7.9%), associated with fatty marrow conversion of the proximal femoral metaphysis.

The Doppler ultrasound pattern of reversed pulsatile flow (RPF) of the portal vein (PV) is strongly associated with high atrial pressure. Tricuspid regurgitation is considered to be the main cause of RPF in patients with chronic heart disease, but the precise pathomechanism of this PV flow pattern has not yet been resolved. We describe for the first time a RPF of the PV in a young patient with a mediastinal haematoma after inadvertent puncture of the subclavian artery. In this patient, transcutaneous echocardiography demonstrated normal valves without any tricuspid regurgitation as well as normal diameters of the cardiac cavities. The RPF of the PV in this patient resolved spontaneously within 7 days. An increased hepatic outflow resistance with transmission of hepatic artery pulsations across arterioportal communications seems the most likely pathomechanism to explain our finding.

Invasion of the major airways is a rare manifestation of respiratory tract involvement by Aspergillus sp. and is seen almost exclusively in immunocompromised patients. We present calcification as a new feature of this condition and its demonstration by ultrasound in a 15-year-old boy with severe neutropenia secondary to aplastic anaemia.

Objective: To diagnose equivocal aplastic anemia(AA) patients by the combination of clinical data and bone marrow scintigraphy., Methods: Bone marrow scintigraphy using 99mTc-sulfer colloid 370-550 MBq was analysed in 47 patients, including 28 cases of AA, 12 of myelodysplastic syndromes(10 MDS-RA and 2 MDS-RAEB) and 7 of AA-PNH syndrome., Results: Pancytopenia, hypercellular bone marrow associated with uneven disappearance of marrow image or focal hematopoiesis were found in 28 AA and 2 of 30 MDS-RA cases. Marrow imaging showed uneven low accumulation of radioactivity in 12 and focal patterns in 18 patients. Normal or high activity and expanded periphery of bone marrow was found in MDS patients (8 RA and 2 RAEB cases). Changes of activity and distribution of bone marrow during the transforming course were noticed in 4 AA-PNH syndrome patients., Conclusions: Patch distribution and focal hemopoiesis is the outstanding features of AA marrow. The imaging features as well as expanded manner and increased activity of bone marrow are helpful to the differential diagnosis, evaluation of clinical status and explanation of marrow aspirate results.

Chimeric mouse-human antigranulocyte monoclonal antibody (ch MAb) against non-specific cross-reacting antigen (NCA-95) was labeled with 99mTc (using a direct method) and 125I (using the chloramine T method), and its binding to human granulocytes and LS-180 colorectal carcinoma cells expressing carcinoembryonic antigen on their surfaces, cross-reactive with anti-NCA-95 chimeric monoclonal antibody, increased in proportion to the number of cells added and reached more than 80% and 90%, respectively. In biodistribution studies, 99mTc and 125I-labeled ch anti-NCA-95 MAb revealed high tumor uptake, and the tumor-to-blood ratio was 2.9 after 24 hours. The tumor-to-normal-organ ratio was also more than 3.0 in all organs except for the tumor-to-kidney ratio. Scintigrams of athymic nude mice confirmed the results of biodistribution studies that showed higher radioactivity in tumor and kidney of the mice administered with 99mTc-labeled ch MAb. A normal volunteer injected with 99mTc-labeled ch anti-NCA-95 antigranulocyte MAb showed clear bone marrow images, and a patient with aplastic anemia revealed irregular uptake in his lumbar spine, suggesting its utility for bone marrow scintigraphy and for the detection of hematological disorders, infections, and bone metastasis.

Unlabelled: Bone marrow is the primary site for many hematologic disorders. To date, however, no suitable bone marrow imaging method has been found. The present study investigates the usefulness of bone marrow immunoscintigraphy using 99mTc-labeled antigranulocyte antibody (anti-NCA-95) in 31 patients with hematologic disorders., Methods: One milligram of antibody labeled with 259-370 MBq 99mTc was injected intravenously, and bone marrow images were taken 4 hr later. We also calculated the uptake ratios of lumbar bone marrow-to-background (L/B) and ilium-to-background (I/B)., Results: Of 15 patients with aplastic anemia, 7 showed diffusely decreased antibody uptake (L/B = 2.3 +/- 0.8, I/B = 3.0 +/- 0.8) compared to control patients (n = 21, L/B = 8.2 +/- 2.5, I/B = 10.3 +/- 3.1) Six patients had both decreased and increased uptake areas and two had normal to slightly increased uptake. Of those patients receiving various types of therapy for aplastic anemia, all but one showed increased or irregular uptake. The degree of antibody uptake in the bone marrow correlated with peripheral blood analyses (hemoglobin, white blood cells, platelets). Of six patients with myelodysplastic syndrome, four had irregular uptake and two diffusely decreased uptake. Four patients with myelogenous leukemia showed normal uptake, whereas two with lymphocytic leukemia had decreased uptake. Patients with iron deficiency anemia, pure red cell aplasia or thalassemia minor exhibited normal uptake with bone marrow expansion., Conclusion: Immunoscintigraphy with antigranulocyte antibody is a useful method for evaluating the bone marrow status of patients with various hematologic disorders.

To specify the validity of bone marrow scanning using a monoclonal anti-granulocyte antibody labelled with 99mTc (BW 250/183) for the functional assessment of haemopoiesis, we compared this method with 52Fe scan in 16 patients with haematological disorders. The examinations were performed using a rectilinear whole-body scanner and the distribution of the two tracers was assessed visually and quantitatively in anatomical bone marrow segments, the spleen and liver. Qualitative comparison showed concordance in the bone marrow distribution of the two tracers in 83% of the segments. Discrepancies were found in six patients with hypoplastic or aplastic marrow. The spleen was visualized in all cases with the 99mTc-Moab, including nine patients without splenic haemopoiesis (i.e. without spleen uptake of 52Fe). The uptake of the two tracers, quantified in bone marrow segments and the spleen, correlated well (P < 0.001), but not in the liver (NS). The correlation between the uptake values for each patient was excellent, except in cases of aplastic bone marrow. In conclusion, bone marrow scanning using a 99mTc labelled anti-granulocyte monoclonal antibody enables functional evaluation of the distribution of haemopoiesis. Limitations include the evaluation of bone marrow aplasia and identification of splenic haemopoiesis, for which 52Fe remains the tracer of choice.

Sonographic determination of spleen mass by ultrasonic tomograph "Toshiba SAL-38AS" is suggested. The results of determination obtained in healthy persons and patients undergoing splenectomy with subsequent weighing of spleen demonstrated high accuracy of the method. Spleen mass in patients with hemoblastosis was revealed to be related closely to the degree of blastosis evaluated by hemo- and myelograms.

High renal uptake on bone marrow scan with indium-111 chloride is often shown in patients with bone marrow abnormality. We evaluated the renal uptake on bone marrow scan in 27 cases with aplastic anemia, 20 cases with myelodysplastic syndrome (MDS) and 10 cases with other diseases. The high renal uptake was observed in patients not only with aplastic anemia but also with MDS. The renal uptake correlated with blood transfusion units, unsaturated iron binding capacity (UIBC), blood pool imaging and bone marrow uptake. The renal uptake correlated with UIBC better than with the blood transfusion units. Following mechanism of the renal uptake is proposed that frequent blood transfusion makes low UIBC, and the low UIBC causes the failure to chelate indium with transferrin. The fast blood clearance of un-chelated indium via kidneys is followed. Hypoplastic bone marrow may also play an important role for the high renal uptake because all patients with the high renal uptake could not be explained by above mentioned mechanisms. Caution should be paid to the scans with the high renal uptake because both aplastic anemia and MDS patients show the high renal uptake.

We report on a female with hypoplastic anemia and abnormally digitalized thumbs who presented with growth failure and novel osseous radiologic abnormalities. In addition to thumb anomalies, abnormalities of the clavicles, ilia, distal sacrum, and coccyx and described.

The diagnosis of systemic haematological disorders is often confirmed by bone marrow biopsy which is usually performed "blind". False-negative results may be obtained because this method is restricted to a small part of the blood-forming organ. Bone marrow scintigraphy has the potential of detecting the distribution of active haematopoietic bone marrow and to localize infiltrative lesions. This case report of a patient with pancytopenia demonstrates that the diagnosis of aplastic anaemia can be confirmed by bone marrow scintigraphy in combination with probe-guided bone marrow biopsy.

An unusual distribution of Tc-99m MDP and Ga-67 citrate in several muscle groups is described in a 14-year-old boy with aplastic anemia and normal renal function at the time of imaging. Although the patient did not develop musculo-skeletal signs or symptoms, a subsequent autopsy revealed widespread muscle calcification.

We describe a patient with viral induced aplastic anemia who developed severe bone pain after treatment with multiple courses of antithymocyte globulin, cyclosporine A, recombinant human granulocyte macrophage colony stimulating factor and deferoxamine. Radiographs and bone biopsy revealed extensive new trabecular bone formation in long bone diaphyses and adjacent periosteal reaction. The effects of hematopoietic growth factors, cyclosporine A and deferoxamine on bone metabolism are reviewed.

A study on the clinical diagnosis of aplastic anemia (AA) was carried out by using 5 different laboratory techniques including bone marrow aspiration and smear. Diagnosis of AA was established in 238 patients. Results with different methods were listed below: (1) Typical picture of AA was found in bone marrow smear of 196 patients. The positive rate was 82.4%. (2) Evaluation of marrow speckles might raise the positive rate to 89.9%. (3) Diagnosis of AA in 103 out of 110 patients was confirmed with bone marrow biopsy. The positive rate was 93.6%. (4) Typical 3-dimensional marrow tissue structure of AA was found in 68 out of 72 patients under scanning electron microscope, with a positive rate of 94.4%. (5) Total body bone marrow gamma-scintigraphy was performed in 56 patients; diagnosis of AA was reconfirmed in 54 patients with a positive rate of 96.4%. (6) 4 or 5 diagnostic techniques were used simultaneously in 30 patients with a positive diagnostic rate of 100%. The methodology of different techniques of bone marrow examination, the typical picture of AA and the causes of missing diagnosis were discussed with certain recommendations on each technique.

13 patients with idiopathic aplastic anaemia in remission for more than 2 years were examined to define the haemopoietic status by means of bone marrow scintigraphy, ferrokinetics and bone marrow culture for haemopoietic progenitor cells. Haemoglobin levels reached the normal range in all these patients although mild neutropenia and thrombocytopenia were still observed in 5 patients. Bone marrow scintigrams using indium-111 showed normal distribution in 2, diffuse low accumulation in 3, patchy distribution in 7, and expanded distribution with patchy uptake in 1 patient. The defective haemopoiesis was also confirmed by ferrokinetic and bone marrow culture studies. The patchy haemopoiesis appears to characterize the residual marrow damage in remission of idiopathic aplastic anaemia.

With 9 kinds of available 99mTc-colloid kits for RES imaging, especially from the aspect of RES imaging of the bone marrow, observations were conducted on pH, the presence of free 99mTcO4(-), labelling efficiency, rabbit blood clearance, the uptake in RES, urinary excretion, as well as the scintigraphy with rabbits and clinical cases. Compared to 198Au-colloid, marrow to liver ratio was found to be about the same, but its uptake to the spleen and liver was greater. In particular its urinary excretion was much more marked than 198Au-colloid, and in clinical use the bladder was more often delineated. This fact seems to suggest that it is desirable to develope a 99mTc-colloid more suitabale for the bone marrow scintigraphy.

This paper reported 4 cases of Fanconi's anemia, which is a rare disease usually seen in children under 10 years of age. Its clinical features comprise familial pancytopenia, hypoplasia of bone marrow, and conspicuous megakaryophthisis, with multiple malformations predominantly occurring in the skeleton and kidney. Some cases may be associated with abnormalities of the chromosome.

Twenty-nine patients with aplastic anemia and 11 patients with myelofibrosis were evaluated with indium-111 chloride bone-marrow imaging, ferrokinetics, and bone-marrow core biopsies. There was good correlation between the erythrocyte cellularity of the marrow and the In-111 bone-marrow scan grades in most patients. In some, the overall scan grade tended to underestimate the erythroid elements because the core biopsy had been taken from the area of the greatest radionuclide concentration on the scan. In patients with aplastic anemia, there was good correlation between the plasma iron clearance t1/2 and the scan grade. Less agreement was found in the comparison between the Fe-59 sacral and organ counts and the red-cell iron utilization. In patients with myelofibrosis, there was poor correlation between the surface counts over the sacrum and the red-cell iron utilization. Plasma iron clearances were abnormally short and were unrelated to the transferrin saturation levels. Eighteen patients were studied several times to evaluate their responses to steroid therapy. In all, there was good correlation between the bone-marrow imaging, the erythrocyte cellularity, ferrokinetics, and the patient's response to therapy. Indium-111 bone-marrow imaging is useful both in evaluating marrow erythroid activity and in following the response to therapy in patients with these diseases.

Bone marrow scintigraphy with indium chloride (111In) was performed in fifty-one patients with the hematological diseases. The results of the investigation were that 1. in all patients, as well as in patients with aplastic anemia, no correlation was there between the degree of the indium chloride accumulation and peripheral blood counts, 2. in patients with aplastic anemia and pure red cell aplasia (PRCA) a tendency to reduction in uptake of indium chloride in bone marrow, 3. in patients with these two good correlation between the degree of indium chloride accumulation and histology of the erythroid bone marrow, but in patients with chronic myelocytic leukemia (CML) and atypical leukemia no correlation between the two, so it seemed unlikely that indium chloride should reflect the effective production of erythrocytes, 4. four patients with leukemia were studied with indium chloride bone marrow imaging two times to evaluate their responses to chemotherapy, and peripheral expansion was no change or reduced in two patients with acute myelocytic leukemia (AML) and one patient with acute lymphocytic leukemia (ALL) who obtained complete remission, but on the other hand, it enlarged in one patient with acute myelocytic leukemia who obtained partial remission, and 5. in two patients with chronic myelocytic leukemia it enlarged up to the ankle joints, which was considerably specific.

Aplastic anaemia affects the entire bone marrow. Current methods of assessment of bone marrow function, like bone marrow biopsy or peripheral blood examination are either invasive or inadequate and cannot be expected to represent fully the changes in the entire bone marrow tissue. This prospective study was undertaken to develop and standardise a new Nuclear Medicine technique called 'Dynamic Bone marrow Imaging'. Eleven patients and ten controls were studied. Serial images of the pelvis were obtained in frame mode following intravenous injection of 185-370 mBq of 99mTc S. Colloid, and an index, called the Bone Marrow Uptake Index (P) was calculated by taking into consideration the time activity curve obtained over the iliac crest. This was followed by static imaging of the entire bone marrow in all cases. It was possible to obtain excellent information regarding topographic distribution of bone marrow as well as detect early changes in bone marrow function following treatment. An attempt was also made to correlate bone marrow cellularity as obtained by bone marrow biopsy with the results of dynamic bone marrow scintigraphy. On the basis of the encouraging results obtained in the present study, the authors feel that dynamic bone marrow imaging is an excellent technique for the objective evaluation of bone marrow in aplastic anaemia. Aplastic anaemia affects the entire bone marrow tissue. Although much progress has been made in the management of this disease, many aspects of it await better understanding. There is almost total lack of knowledge regarding the distribution of functioning marrow in various phases of aplastic anaemia, such as in relapse and remission. Current methods of assessment of marrow function rely mainly on bone marrow biopsy and peripheral blood examination. Bone marrow biopsy is invasive and cannot be expected to represent fully the changes in the entire tissue. Changes in peripheral blood picture lag behind the changes in the bone marrow. Thus, there is a need for an investigation which is safe, simple, sensitive, non invasive and capable of assessing the global function of bone marrow. Radio-nuclide imaging of bone marrow requires labelling of one or more components of this widely dispersed tissue. The reticuloendothelial and erythropoietic components can be labelled with radio-colloids and radio-iron respectively. Experimental studies have shown that the reticuloendothelial and erythropoietic elements are invariably found together in the marrow and have similar distribution. This report is based on a prospective study of bone marrow function in patients with aplastic anaemia, using 99mTc. Sulphur colloid.

Twenty-one patients (12 male, nine female) with diverse causes of aplastic anemia underwent indium chloride in 111 bone marrow scintigraphy. Good correlation of the scan interpretation with other clinical values, including the peripheral blood counts and bone marrow biopsy, was found in 25 of the 26 studies. These encouraging results with a noninvasive procedure that permits evaluatin of the entire bone marrow organ suggest a broader use for indium bone marrow scintigraphy in aplastic anemia.