Leukoerythroblastosis in a Young Child with Severe Malaria and Superimposed Gram Negative Infection.

Background: Leukoerythroblastosis, a non-specific and often short-lasting response of the bone marrow to different diseases such as malignancies or infections, is characterized by the presence in the peripheral blood of immature red and white cells. Methods: We present a case of leukoerythoblastosis occurring in a 24 months old Mozambican girl, in the context of a severe malaria episode and an associated urinary tract infection. Peripheral blood smear was used for diagnosis of malaria and leukoerythroblastosis. Enterobacter cloacae isolation and antibiotic susceptibility testing were performed by conventional microbiology. Results: Peripheral blood smear was positive for Plasmodium falciparum and showed a leukoerythroblastosis with red cell anisopoikilocytosis and left shifted neutrophils. Urine culture confirmed the presence of a multi-resistant E. cloacae. Treatment of underlying conditions resolved the leukoerythroblastic reaction. Conclusions: Leukoerythroblastosis may be related to different infectious diseases and may also appear in the context of severe malaria. Bacterial superinfection needs to be investigated.

Response after treatment with pembrolizumab in a patient with myelophthisis due to melanoma: the role of checkpoint inhibition in the bone.

BACKGROUND: Myelophthisis due to melanoma is a rare phenomenon. Treatment strategies for patients with this serious complication of malignancy have not been well documented, and none have previously reported efficacy of immune checkpoint inhibition. Since bone metastases are not measurable lesions per standard response criteria, the efficacy of immune checkpoint inhibition in the bones is also not well described. CASE PRESENTATION: We describe a patient with widespread melanoma metastases involving the bone marrow causing myelophthisis and pancytopenia who responded to immune checkpoint inhibition with the anti-programmed cell death-1 (PD-1) inhibitor pembrolizumab. CONCLUSIONS: This is the first report to our knowledge of disease response to immune checkpoint inhibition in a patient with myelophthisis. Clinical trials have recently emerged describing the efficacy of PD-1 inhibition for disorders regularly involving the bone marrow, such as hematologic malignancies, suggesting the importance of better understanding the bone marrow as an immunologically active compartment. Clinicians should be aware that immune checkpoint inhibition alone may be effective in treating malignancy involving the bone marrow, even in cases of extensive involvement resulting in pancytopenia due to myelophthisis from a solid tumor as our case suggests.

Ghosal hematodiaphyseal dysplasia: a rare cause of a myelophthisic anemia.

Ghosal hematodiaphyseal dysplasia syndrome (GHDD) is a rare clinical syndrome characterized by increased bone density and a severe, myelophthisic anemia. Few cases have been reported worldwide and there are no detailed descriptions of the associated hematologic abnormalities and long-term clinical outcomes after treatment. Here, we report two siblings with GHDD who were successfully treated with chronic, low dose, corticosteroid therapy. Although GHDD is uncommon, these cases illustrate the need to consider GHDD in patients with anemia and bone dysplasia and the use of chronic, low-dose steroid therapy.

Effects of weiganli on the hemopoietic function of the myelosuppressed anemic mice.

OBJECTIVE: To study the effect of weiganli ([Chinese characters: see text]) on bone marrow hemopoiesis. METHODS: The effects of weiganli on the peripheral blood picture and the number of bone marrow nucleated cells (BMCs) were observed in myelosuppressed anemic model mice, and the effects of weiganli on the growth of colony forming unit-granulocyte macrophage (CFU-GM), colony forming unit-erythroid (CFU-E), burst forming unit-erythroid (BFU-E), colony forming unit-megkaryocyte (CFU-Meg) were investigated by in vitro cell culture technique. The hemopoietic stem cells (HSCs, c-kit+) in bone marrow were double stained with fluorescent antibody PE-C-Kit and FITC-CD45, and the HSCs (c-kit+) were counted by flow cytometer with CD45/SSC (side scatter) gating. RESULTS: Peripheral blood cell counts and the number of BMCs were significantly improved after weiganli administration; and bone marrow hemopoietic stem/progenitor cells were significantly increased. CONCLUSION: Weiganli can effectively promote the recovery of hemopoietic function in the myelosuppressed anemic mice.

Lenalidomide therapy in myelofibrosis with myeloid metaplasia.

We present results of 2 similarly designed but separate phase 2 studies involving single-agent lenalidomide (CC-5013, Revlimid) in a total of 68 patients with symptomatic myelofibrosis with myeloid metaplasia (MMM). Protocol treatment consisted of oral lenalidomide at 10 mg/d (5 mg/d if baseline platelet count < 100 x 10(9)/L) for 3 to 4 months with a plan to continue treatment for either 3 or 24 additional months, in case of response. Overall response rates were 22% for anemia, 33% for splenomegaly, and 50% for thrombocytopenia. Response in anemia was deemed impressive in 8 patients whose hemoglobin level normalized from a baseline of either transfusion dependency or hemoglobin level lower than 100 g/L. Additional treatment effects in these patients included resolution of leukoerythroblastosis (4 patients), a decrease in medullary fibrosis and angiogenesis (2 patients), and del(5)(q13q33) cytogenetic remission accompanied by a reduction in JAK2(V617F) mutation burden (1 patient). Grade 3 or 4 adverse events included neutropenia (31%) and thrombocytopenia (19%). We conclude that lenalidomide engenders an intriguing treatment activity in a subset of patients with MMM that includes an unprecedented effect on peripheral blood and bone marrow abnormalities.

Coeliac disease presenting with a leuco-erythroblastic anaemia.

A 49-year-old Irishman presented as an emergency with watery diarrhoea and a leuco-erythroblastic anaemia. Investigations showed that he had coeliac disease but no evidence of bone marrow infiltration. His leuco-erythroblastic picture disappeared on treatment with iron and folic acid.

Leucoerythroblastic anaemia in carcinoma of ovary.

The survey of cases of ovarian carcinoma referred to the Radiotherapy Centre at Sheffield between 1956 and 1965 was undertaken, which revealed one case of leucoerythroblastic anaemia as a complication. This patient also had a demonstrable bony metastases, which appeared eight years after the apparent cure of the diasease-a most unusual feature of carcinoma of ovary.