Glomerular diseases associated with chronic graft-versus-host disease after allogeneic peripheral blood stem cell transplantation: case reports.

Chronic graft-versus-host disease (cGVHD) is the major complication following allogeneic stem cell transplantation (allo-SCT). Nephrotic syndrome (NS) and other types of glomerulonephritis have been proposed to be the very rare forms of renal cGVHD. From 1991 to 2011, 253 patients underwent allo-SCT at our center. We report here 4 cases (1.6%) presenting with varieties of glomerular manifestations associated with cGVHD. The first case was typical NS. The renal pathology showed membranous nephropathy (MN). The second case was also MN, but this patient also had the pathology of focal segmental glomerulosclrosis (FSGS) and acute tubular necrosis (ATN). The third case showed lupus nephritis-like glomerular lesions with a high anti-nuclear antibody (ANA) titer. The fourth case presented with rapidly progressive glomerulonephritis (RPGN)-like symptoms. The kidney histology in this case was not available. The patient responded well to immunosuppressive therapy, but NS later recurred. Therefore, overt glomerular diseases after allo-SCT in Thai patients are not very rare. Monitoring urinalysis during withdrawal of immunosuppressive drugs and also during follow-up of patients with cGVHD may be considered.

Protein C, protein S and von Willebrand factor levels correlate with bleeding symptoms: a population-based study.

Although natural anticoagulant deficiencies are the established causes of thrombosis, their roles in bleeding are not fully studied. The objective is to correlate haemostatic factors with haemorrhagic symptoms quantified by a standardized questionnaire. Adult subjects were recruited from Bangkok and nearby provinces as part of routine health surveys/checkups. The validated MCMDM-1VWD form was used to assess their bleeding symptoms. At the same time, von Willebrand factor (VWF) activity, free protein S levels and protein C activity were measured. There were 5196 individuals. The mean age was 44.3 years (range 15-99) and 41% were male subjects. The mean bleeding score was -0.28 and 95% of subjects had scores between -2 and +2. The scores were lower in female subjects than in male subjects (-0.35 vs. -0.16, P < 0.001). Bleeding scores correlated negatively with age, VWF and protein C activities (Spearman's ρ-0.258, -0.091 and -0.098, respectively, all P < 0.001), but did not significantly correlate with protein S levels. Using multivariate analysis, female gender, VWF below 100 IU dL(-1), protein C below 100 IU dL(-1) and protein S over 150 IU dL(-1) significantly related to high (≥3) bleeding scores (adjusted odds ratio 1.95, 1.83, 1.56 and 2.84, P = 0.001, 0.001, 0.039 and 0.017, respectively). These findings may suggest interacting roles of VWF and natural anticoagulants in modifying bleeding symptoms.

Treatment of myelodysplastic syndrome with low-dose human granulocyte colony-stimulating factor: a multicenter study.

The objective of this study was to determine the hematopoietic effects and toxicity of low-dose granulocyte colony-stimulating factor (G-CSF) in myelodysplastic syndrome (MDS) patients with neutropenia. Recombinant human G-CSF (Lenograstim) was administered by daily subcutaneous injection with an initial dosage of 0.5 microg/kg per day for 2 weeks. Patients not responding to the initial dosage received the escalated dosage, 1 to 2 microg/kg per day for 2 weeks. Eligibility criteria were the following: French-American-British disease classification subtype refractory anemia (RA), refractory anemia with ringed sideroblasts (RARS), or refractory anemia with excess blasts (RAEB) with an absolute neutrophil count (ANC) of <1.5 x 10(9)/L. Criteria indicating response to treatment were ANC of >1.5 x 10(9)/L and doubling of ANC on at least 2 occasions. Thirty-two MDS patients were recruited from 6 university hospitals. Eighteen patients had RA, 4 had RARS, and 10 had RAEB. Median age was 56.4 years (range, 28-87 years). Twenty-six patients (81.2%) had an increase in ANC from a median of 0.94+/-0.35 x 10(9)/L to 4.24+/-3.78 x 10(9)/L. Three of 6 patients who did not respond to the initial dosage responded to the escalated dosage of 1 microg/kg per day. Eighteen (81.8%) of 22 patients with RA or RARS responded compared with 8 (80%) of 10 patients with RAEB. The response rates in patients with ANCs of <0.5 x 10(9)/L. 0.5 to <1.0 x 10(9)/L, and 1.0 to 1.5 x 10(9)/L were 80%, 70%, and 88.2%, respectively. The side effects were minimal. No significant changes in hemoglobin levels or platelet counts were observed. In conclusion, low-dose G-CSF administered by subcutaneous injection is well tolerated and effective in improving neutropenia in MDS patients.

A predictive model for life-threatening neutropenia and febrile neutropenia after the first course of CHOP chemotherapy in patients with aggressive non-Hodgkin's lymphoma.

The purpose of this study was to develop a model for predicting the occurrence of life-threatening neutropenia (LN, ANC < or = 0.5 x 10(9)/l) and febrile neutropenia (FN, an ANC < 0.5x10(9)/l in association with a body temperature of > or = 38.3 degrees C) after the first cycle of CHOP therapy in patients newly diagnosed with aggressive NHL. One hundred and forty-five patients, aged > or = 15 years, with newly diagnosed diffuse mixed, diffuse large-cell or large-cell immunoblastic lymphoma (IWF categories, F, G, H), who had been treated with CHOP at King Chulalongkorn Memorial Hospital between June 1994 and December 1998, were entered into the study. The criteria for eligibility included complete work-up for baseline evaluation, treatment with standard CHOP chemotherapy, at least one complete blood count performed during days 8-14 post-treatment or if at any time the patients experienced a BT of > or = 38.3 degrees C and were not treated with any colony-stimulating factors (CSFs). The median age of the patients was 47 years (range, 17-78). Forty-eight percent of the patients were in stage III/IV, 36% had ECOG performance status (PS) II-IV, 30% had > or = 2 extranodal diseases, 59% had serum LDH > 1 x normal and 23% had bone marrow involvement. The frequencies of patients in the low-, low-intermediate, high-intermediate and high risk groups according to the international index were 29%, 28%, 17% and 26%, respectively. Thirty-nine percent of the patients had LN at nadir and 33% developed FN after the first course of CHOP. By using stepwise logistic regression analysis, the pretreatment variables independently predictive of the LN at nadir and the FN were serum albumin concentration of < or = 3.5 g/dl, serum LDH > 1 x normal and whether there was bone marrow involvement of lymphoma at presentation. The model, based on the incorporation of these three factors, identified three risk groups of patients with a predicted probability of developing LN at nadir of 81.5% (95% CI, 68.5-90.7) (high risk), 23.9% (95% CI, 12.6-38.8) (intermediate risk) and 4.4% (95% CI, 0.5-15.1) (low risk). The predicted rate of FN in the three groups were 72.2% (95% CI, 58.4-83.5), 17.4% (95% CI, 7.8-31.4) and 2.2% (95% CI, 0.05-11.8), respectively. In conclusion, our model could be used as a means to identify patients with newly diagnosed aggressive NHL, treated with CHOP, who are at high risk (> or = 50% probability) of developing post-first course LN and FN, in whom CSF and/or antibiotic prophylaxis might be indicated.

CHOP versus CHOP plus ESHAP and high-dose therapy with autologous peripheral blood progenitor cell transplantation for high-intermediate-risk and high-risk aggressive non-Hodgkin's lymphoma.

The purpose of the study was to compare conventional cyclophosphamide/doxorubicin/vincristine/prednisolone (CHOP) chemotherapy with CHOP (3 courses) plus etoposide/methylprednisolone/high-dose cytarabine/cisplatin (ESHAP), high-dose therapy (HDT), and autologous peripheral blood progenitor cell transplantation (PBPCT) as front-line treatment for poor-prognosis aggressive non-Hodgkin's lymphoma (NHL). Between May 1, 1995, and April 30, 1998, 58 patients, aged 15-55 years, newly diagnosed with poor-prognosis aggressive NHL (category F-H by the Working Formulation) were enrolled. According to the age-adjusted international prognostic index, 65% of the patients were high-risk cases and 35% made up the high-intermediate group. After 3 courses of CHOP, 25 of 48 patients were randomized to continue with CHOP, and 23 were randomized to receive 2-4 cycles of ESHAP followed by HDT and PBPCT. There was no significant difference in the rate of complete remission between the two groups (36%, 95% confidence interval [CI]: 18%-57% in CHOP vs. 43%, 95% CI: 23%-65% in ESHAP/HDT) (P = 0.77). With a median follow-up duration of 39 months, the 4-year failure-free survival (FFS) was superior in the ESHAP/HDT group (38%, 95% CI: 18%-58% vs. 15%, 95% CI: 4%-32%) (P = 0.04). The disease-free survival was marginally different in favor of the ESHAP/HDT arm (90%, 95% CI: 47%-98% vs. 37%, 95% CI: 7%-69%) (P = 0.06). The 4-year overall survival between the two treatment arms was comparable (51%, 95% CI: 28%-70% for ESHAP/HDT vs. 30%, 95% CI: 13%-48% for CHOP) (P = 0.25). Treatment-related mortalities were not significantly different between both groups (17%, 95% CI: 5%-39% for ESHAP/HDT vs. 8%, 95% CI: 1%-26% for CHOP) (P = 0.41). However, only 61% of the patients assigned to the ESHAP/HDT arm underwent HDT and PBPCT. As compared with CHOP, the corporate regimen of CHOP/ESHAP/HDT seems to improve the FFS in patients with newly diagnosed, poor-prognosis aggressive NHL.

A prospective randomized study of Chop versus Chop plus alpha-2B interferon in patients with intermediate and high grade non-Hodgkin's lymphoma: the International Oncology Study Group NHL1 Study .

UNLABELLED: The addition of a brief alpha interferon regimen to each CHOP induction cycle, plus one year of alpha interferon thrice weekly maintenance therapy, has no early effect on response rates or survival in patients with Intermediate or High grade cell NHL. BACKGROUND: The CHOP (Cyclophosphamide, Adriamycin. Vincristine, Prednisone) regimen is the most widely used first-line therapy for patients with Intermediate or High Grade (IG/HG) non-Hodgkin's lymphoma (NHL). Alpha 2b interferon (INF) enhances response rates and improves survival in low-grade NHL. The International Oncology Study Group (IOSG) conducted a prospective randomized study comparing CHOP alone or combined with INF in patients with IG/HG-NHL. The primary study aim was to compare the objective response rates in these patient cohorts. PATIENTS AND METHODS: Patients with a confirmed diagnosis of measurable NHL of International Working Formulation (IWF) groups D to H histology were randomized to receive CHOP alone or CHOP with 5Mu INF s.c. for 5 days on days 22 to 26 of each 28 day cycle with INF 5 million units (Mu) given three times per week subcutaneously for 52 weeks in those patients who responded to CHOP plus INF. RESULTS: The overall response rates were equivalent in both groups: CHOP alone (214 patients) 81% (complete 55%, partial 26%); CHOP plus INF (221 patients) 80% (complete 54%, partial 26%). At 36 months, the actuarial survival rate was equivalent in both groups. CONCLUSIONS: There is no apparent early advantage in terms of response or survival conferred by adding the study INF regimen to CHOP therapy for patients with IG/HG-NHL.

Quality of life in Thai patients after bone marrow and peripheral blood stem cell transplantation: a comparison study with patients treated with conventional chemotherapy.

This study of adult patients of Thai cultural background compared the quality of life (QOL) of patients undergoing bone marrow and peripheral blood stem cell transplantation (BMT/PBSCT) with that of patients treated with conventional chemotherapy (CT). The study population comprised 59 patients who were transplant recipients (29 allogeneic, 29 autograft, and one syngeneic BMT/PBSCT) and 24 patients treated with CT for various hematologic malignancies. The participants completed a 50-item structured QOL questionnaire sent by mail. No significant differences were found between the two patient groups regarding their psychological, social, and treatment-related well-being or their overall QOL; all scores were above 75% of the total. The mean score regarding the physical domain, however, was significantly lower in BMT/PBSCT patients. Areas of highest concern in both groups were sexuality and financial burden. Happiness, functional ability, financial burden, and the degree of familial acceptance were the most important predictors of the self-rated QOL in BMT/PBSCT patients. Seventy-five percent of BMT/PBSCT patients would be willing to undergo the procedure again under the same circumstances. These results indicated that the QOL of Thai patients undergoing BMT/PBSCT was in general satisfactory and comparable to patients treated with conventional CT.

The effects of green pit viper (Trimeresurus albolabris and Trimeresurus macrops) venom on the fibrinolytic system in human.

Green pit viper (Trimeresurus albolabris and Trimeresurus macrops) venom was found to have a thrombin-like effect in vitro but cause a defibrination syndrome in vivo. The effects of venom on fibrinolytic system have not been well characterized. This knowledge can help to define the roles of antifibrinolytic therapy, give insights in fibrinolytic system regulation and potentially lead to identification of a new profibrinolytic agent from this venom. Forty-six cases of green pit viper bites were studied for various coagulation and fibrinolytic parameters and correlated with serum venom levels measured by ELISA. Fibrinolytic system activation is very common as indicated by low plasminogen (50%), low antiplasmin (56.5%) and elevated fibrin-fibrinogen degradation products (FDPs, 97.4%) levels. FDP test is very sensitive and a normal level is useful for exclusion of systemic envenomation. In contrast to some other models of defibrination syndrome, such as Russell viper (Daboia russelli siamensis), elevation of plasminogen activator activity (PA) was found indicating a hyperfibrinolytic state. Definite increase in tissue-type plasminogen activator (t-PA) antigen (p = 0.00075) with a modest elevation of its inhibitor plasminogen activator inhibitor-1 (PAI-1) (p = 0.27) probably contributes to this effect. This supports the idea that the balance between plasminogen activators and inhibitors can determine fibrinolytic responses in pathologic states. Fibrinopeptide A levels were markedly elevated (68.43 +/- 51.57 ng/ml in cases and 2.83 +/- 3.80 ng/ml in control, p < 0.0001) and correlated well with clinical severity suggesting that the fibrin deposition from the thrombin-like effect is the main mechanism of fibrinolysis. Therefore, antifibrinolytic agents probably have no role in treatment. However, the components of green pit viper venom that have these profibrinolytic effects in human are interesting and should be further identified.

Myelodysplastic syndromes in Thailand: a retrospective pathologic and clinical analysis of 117 cases.

To gain more insight into the understanding of myelodysplastic syndromes (MDS) as they occur in Thailand, a retrospective clinicopathologic analysis was conducted in patients (age > 15 years) diagnosed as MDS from January 1992 to December 1996 at the five major medical centers in various geographic regions of the country. The central reviewers independently examined the bone marrow and peripheral blood smears of all the patients and classify the disease according to the French-British-American (FAB) classification. There were a total of 117 eligible patients. The median age of the patients was 56 years (range 16-86). The male:female ratio was 1:1. Thirty-two percent of the patients were younger than 40 years. The frequency of the FAB subtypes was RA/RARS, 54.7; RAEB, 23.1; CMML, 9.4; and RAEB-T, 12.8%. Anemia was the most common symptom presenting in 84.6% of the patients. In the 34 patients in whom the cytogenetics in the bone marrow were analysed, 44.1% revealed abnormalities. Of these, monosomy 7 and trisomy 8 were the most common aberration, each being detected in 26.7% of the patients. Transfusions were the main therapeutic modality in 80% of the patients. Kaplan-Meier analysis revealed a 5 year survival rate of 29% for the whole group with a median survival of 24 months. Twenty-five percent of the patients had progressed to acute myelogenous leukemia (AML) with a median time to disease-progression of 23 months. The median survival for RA/RARS, RAEB, CMML and RAEB-T were 58.4, 19.9, 10.7 and 8.7 months, respectively (P < 0.001). The stepwise Cox regression analysis revealed the percentage of blasts in the bone marrow as the only parameter significantly associated with survival and disease progression. On comparison with data from other countries, the age of Thai patients with MDS is considerably lower than the western population but is comparable to other asian countries. The distribution of the FAB subtypes and the survival of the patients are similar. The major prognostic features, however, lie in the percentage of blasts in the bone marrow rather than the degree of the observed cytopenia.

The role of serum ferritin in the diagnosis of iron deficiency anaemia in patients with liver cirrhosis.

OBJECTIVES: To determine the diagnostic values of serum ferritin and other conventional laboratory tests in the diagnosis of iron deficiency anaemia in patients with liver cirrhosis. DESIGN: Cross-sectional study for diagnostic tests. SETTING: University hospital. SUBJECTS: Seventy-two consecutive patients with liver cirrhosis in whom the haemoglobin level was less than 13.0 g dL(-1) for men and 12.0 g dL(-1) for women. The diagnosis of liver cirrhosis was based on characteristic clinical and hepatic ultrasonographic findings. MAIN OUTCOME MEASURES: By using absence of bone marrow iron as the standard criterion, the diagnostic powers of mean corpuscular volume, transferrin saturation, serum ferritin and the presence of hypochromic red cells in the diagnosis of iron deficiency were compared by analysing the likelihood ratios, the area under the receiver operating curves (ROC) and the stepwise logistic regression associated with each test. RESULTS: Twenty-nine patients (40.3%) demonstrated no stainable iron in the bone marrow. The likelihood ratios, the area under the ROC and the stepwise logistic regression indicated that serum ferritin was the most powerful test predictive of iron deficiency. Other tests added little further diagnostic values. The likelihood ratios associated with the serum ferritin levels were as follows: <50 microg L(-1), 22.3; 51-200 microg L(-1), 1.5-1.8; 201-400 microg L(-1), 1.0; >400 microg L(-1), <1. These results indicate that serum ferritin level <50 microg L(-1) depict a very high probability of iron deficiency anaemia (0.83-0.99) irrespective of the patient's pre-test probability. CONCLUSION: Serum ferritin is the most powerful noninvasive test for the diagnosis of iron deficiency anaemia in patients with liver cirrhosis. It should be the primary test of choice in patients suspected of having the disease. When the level was less than 50 microg L(-1), iron supplement may be prescribed without necessitating bone marrow aspiration.

Anemias in Thai patients with cirrhosis.

Anemia is a frequent complication in patients with cirrhosis. Only one study has been previously reported regarding the etiology of anemias in Thai cirrhotic patients. The diagnosis of iron deficiency in the study however was not based on standard criteria. Herein we report the frequency and hematological manifestations of various causes of anemias diagnosed by using gold standard criteria in 72 consecutive Thai cirrhotic patients. The diagnosis of cirrhosis was based on the characteristic clinical features and the ultrasonographic findings. The median age of the patients was 49 years; male:female was 1:1.3. The mean hemoglobin value was 8.3 g/dl and the mean MCV was 96.6 fl. Most patients revealed macrocytosis, normal WBC count and mild thrombocytopenia. Iron deficiency, defined as absent bone marrow iron stores, was the most common anemia found in 40% of the patients while folate deficiency, diagnosed when red cell folate was < 160 ng/ml packed RBC, was documented in 10% of the patients. Megaloblastosis, hemolysis and anemia of chronic disease was found in 4%, 28% and 13% of the patients, respectively. Folate deficiency was significantly more common in the alcoholic patients (P = 0.01). Iron deficiency was thus the most common anemia in Thai patients with cirrhosis. The frequency of folate deficiency was not rare and the rate was comparable to data reported from western countries in spite of the Thai diet being relatively rich in folates.

Non-Hodgkin's lymphomas in Thailand. A retrospective pathologic and clinical analysis of 1391 cases.

BACKGROUND: Geographic variations in the histopathologic pattern of non-Hodgkin's lymphoma (NHL) are well documented. Insight into this epidemiologic data might shed light on the underlying etiology. Currently, there is a paucity of information regarding the pattern of NHL occurring in Thailand and Southeast Asia. The current nationwide study was undertaken to obtain a clearer definition of the disease among Thais and to compare this information with data from other Asian and Western countries. METHODS: A retrospective analysis of histopathologic subtypes and clinical features was conducted in 1391 patients (age > or = 15 years) with NHL, who were treated at 6 major medical centers in 4 representative areas of Thailand. One hundred and thirty unselected cases were immunohistochemically studied. The reports from other countries used for comparison were identified through a computerized search on MEDLINE. RESULTS: Of the total cases studied, follicular lymphomas constituted 3.8% and diffuse lymphomas 91.4%. The frequency of low and intermediate grade (including large cell immunoblastic, using the Working Formulation) were 12.8% and 72.9%, respectively. The most common histologic subtype was the diffuse large cell and large cell immunoblastic entity, which constituted 39.9% of the cases studied. The rate of small lymphocytic and diffuse small cleaved subtypes were higher than in the United States. Of the 130 cases studied, T-cell lymphoma comprised 16.1%, which was much less than in Japan, China, or Taiwan. CONCLUSIONS: The histopathologic pattern of NHL in Thailand was characterized by the features noted for Asia, i.e., a low rate of the follicular entity and a preponderance of the diffuse aggressive subtypes. In addition, the frequency of the small lymphocytic and diffuse small cleaved subtypes were increased compared with the Western population and the rate of T-cell lymphoma appeared to be less than in Far East Asia.

Neurological manifestations in patients with malignant histiocytosis.

Of the 67 patients who were diagnosed with malignant histiocytosis at the Department of Medicine, Chulalongkorn Hospital, from January 1981 to May 1992, seven (10%) were found to have neurological manifestations. In five patients, neurologic diseases were the presenting clinical features. In the remaining two patients, the disease manifested at the time of relapse. Leptomeningeal infiltration, the most common pattern of involvement, was found in five patients. Involvement of the spinal cord was noted in three patients and cerebral parenchyma in two individuals. Two patients demonstrated peripheral neuropathy. Rapid clinical deterioration was observed in all patients after documentation of neurological diseases with a mean survival of 1.8 months. It is concluded that the frequency and pattern of neurological manifestations in patients with malignant histiocytosis are comparable to patients with non-Hodgkin's lymphoma. However, most patients with malignant histiocytosis manifested the neurological symptoms as an initial presentation while in non-Hodgkin's lymphoma, neurologic diseases usually manifest at the time of relapse or disease progression.

Echocardiographic features in patients with beta thalassemia/hemoglobin E: a combining effect of anemia and iron load.

Hemoglobin concentration, iron parameters and cardiac status of 25 patients with beta thalassemia/hemoglobin E disease were measured. Two-dimensional echocardiography was employed to evaluate chamber size, ejection fraction and muscle fiber shortening. The seventeen patients who had a mean hemoglobin concentration of less than 7.7 g/dl were found to have significantly larger cardiac chambers and higher cardiac output than the patients with higher hemoglobin concentrations. A statistically significant difference in ejection fraction and per cent of fractional shortening of cardiac muscle fibers was not found. A comparison of subjects with ferritin concentrations above and below 1,500 micrograms/L showed no significant difference in chamber sizes, ejection fraction and in per cent fractional shortening. However, a comparison of 14 patients with transferrin saturation > or = 80 per cent with those less than 80 per cent showed a significant decrease in both ejection fraction and fiber fractional shortening in the more highly saturated subjects. By using stepwise multiple regression analysis, the hemoglobin level was shown to significantly determine cardiac enlargement, while ejection fraction and percentage fractional shortening were significantly associated with transferrin-iron saturation. Thus, anemia leads to chamber enlargement, while high transferrin iron saturation is associated with cardiac muscle dysfunction. The findings in the individual patient reflect the integration of these two effects.

POEMS syndrome: a case with proliferative vasculopathy and a review of cases Thailand.

A Thai male, aged 36 yrs, presented with chronic progressive sensorimotor polyneuropathy and mixed osteosclerotic and lytic lesions at the lumbar vertebrae (L). Generalized hyperpigmentation, bilateral gynecomastia, small testes, hepatomegaly, lymphadenopathy and ascites were observed. Computerized tomography of the lumbosacral spines revealed osteosclerotic changes at L3,4,5 with spiculated bony proliferation at the transverse processes and posterior components of the spines. Histopathological examination of the lesion was compatible with plasmacytoma. Proliferative vasculopathy was demonstrated in plasmacytoma. Serum immunofixation showed an IgM monoclonal gammopathy. Further investigation demonstrated secondary adrenal insufficiency with low level of plasma testosterone. The patient responded well to local radiotherapy and intermittent chemotherapy with melphalan and prednisolone. The histopathological findings of proliferative vasculopathy suggested the important role of capillary leakage as a factor responsible for systemic manifestations in the POEMS syndrome. The reported cases of this syndrome among Thais are summarized.

Obstructive uropathy due to extramedullary haematopoiesis in beta thalassaemia/haemoglobin E.

An 18 year old woman with beta thalassaemia/haemoglobin E developed a large pelvic tumour resulting in bilateral obstructive uropathy. Technetium-99m sulphur colloid marrow image, computed tomographic scan of the abdomen and needle biopsy of the mass confirmed the diagnosis of extramedullary haematopoiesis. Although radiation is the treatment of choice for decompression, the mass in this patient did not respond satisfactorily due to its multiple area of tumour autoinfarction. Obstructive uropathy due to extramedullary erythropoiesis has not to our knowledge been previously described.