Clinical and molecular characteristics and prognosis of classical hairy cell leukemia and hairy cell leukemia variant / 中华内科杂志

Objective: To evaluate the clinical characteristics, treatment response, and outcomes in patients with classical hairy cell leukemia (cHCL) and HCL variant (HCL-V). Methods: This is a retrospective case series study. Between January 2011 and December 2021, clinical data of 30 patients newly with diagnosed HCL at Peking Union Medical College Hospital were analyzed. The main outcome measures include clinical characteristics, treatment efficacy and survival. The Kaplan-Meier method was used for survival analysis. Results: Twenty-one cases of cHCL and 9 cases of HCL-v were included. The median age at diagnosis was 55.5 (range, 30-86) years, with the ratio of male to female 2.75∶1. The main clinical manifestations included fatigue in 11 cases (36.7%), abdominal distension in 7 cases (23.3%), and infection in 4 cases, while 8 cases were asymptomatic. Splenomegaly was reported in 24 cases (80.0%), including 7 (23.3%) with megalosplenia. The white blood cell count, lymphocyte count, and the proportion of peripheral hairy cells in HCL-v group were significantly higher than those in cHCL group, whereas the development of anemia, thrombocytopenia, and monocytopenia in cHCL group was more remarkable than that in HCL-v group (all P<0.05). The BRAF-V600E gene mutation was detected only in cHCL patients (11/14 vs. 0/9, P<0.001). In terms of immunophenotype, the expression of CD25, CD103, CD123 and CD200 in cHCL group (20/20, 20/20, 4/7, 7/17) were all stronger than those in HCL-v group (3/9, 7/9, 0/4, 2/8). Twenty-two patients were treated, of which 13 cases (12 cases of cHCL and 1 case of HCL-v) with cladribine, and 9 cases (4 cHCL and 5 HCL-v) with interferon. Complete remission rate and overall response rate were comparable between cladribine and interferon treatment groups (both P<0.05). The median follow-up time was 31 (range, 1-125) months, and the median overall survival (OS) of the entire group was 125 months. The 5-year OS rate in HCL-v patients represented a trend of inferior (50.0% vs. 95.0%, P=0.207). Conclusions: The clinical features of HCL are unspecific, which includes fatigue, splenomegaly and recurrent infection. The clinical features, immunophenotype, treatment response and prognosis of HCL-v are different from those of cHCL. BRAF-V600E gene mutation is suggested as a key marker for differential diagnosis. Cladribine is recommended as front-line regimen of cHCL patients with satisfactory efficacy and prognosis. Conversely, response and clinical outcome in HCL-v patients still need to be improved.

Tinospora cordifolia as an adjuvant drug in the treatment of hyper-reactive malarious splenomegaly--case reports.

BACKGROUND & OBJECTIVE: The effect of aqueous extract of Tinospora cordifolia, an immunomodulator with antimalarial activity along with chloroquine was studied in the treatment of three cases of hyper-reactive malarious splenomegaly in District Hospital, Daltonganj town, Jharkhand, India. These cases were partial/slow responders to the conventional antimalarial drug chloroquine. METHODS: Aqueous extract of T. cordifolia (500 mg) was added to chloroquine (CQ) base (300 mg) weekly and CQ prophylaxis was observed up to six months. Improvement was gauzed by measuring spleen enlargement, Hb, serum IgM and well-being in three cases of hyper-reactive malarious splenomegaly. RESULTS: Addition of extract of T. cordifolia for the first six weeks to chloroquine showed regression of spleen by 37-50% after six weeks and 45-69% after six months from the start of treatment. Likewise decrease in IgM and increase in Hb as well as wellbeing (Karnofsky performance scale) were observed. CONCLUSION: The results of the present study paves a new sight in the treatment of hyper-reactive malarious splenomegaly, however, large-scale trial is required to confirm the beneficial effect of T. cordifolia extract in combination with chloroquine.

Ocurrencia de leucemia mieloide crónica y linfoma no Hodgkin en un mismo paciente: caso clínico / Simultaneous occurrence of chronic myeloid leukemia and non Hodgkin lymphoma: clinical case

Chronic myeloid leukemia is a myeloproliferative disorder caused by a clonal disturbance of the trunk cell and the accumulation of granulocytic series in the marrow, blood and other organs. We report a 63 years old male, carrier of a chronic myeloid leukemia whose clinical condition was complicated by the appearance of a T cell lymphoma. He was subjected to chemotherapy, that reduced the size of adenopathies and improved his general condition. Further studies are required to determine if there is a relationship between these two clinical entities

Bone mineral density in children and adolescents with hepatosplenic mansonic schistosomiasis and esophageal varices who underwent splenectomy and ligature of the left gastric vein

Twenty eight children and adolescents from 7 to 19 years of age, suffering from hepatosplenic mansonic schistosomiasis and bleeding esophageal varices were evaluated for bone mineral density (BMD), before undergoing medical and surgical treatment. The surgical protocol was splenectomy, autoimplantation of spleen tissue into a pouch of the greater omentum and ligature of the left gastric vein. Twenty one patients were evaluated after a follow up from two to nine years post surgical treatment. The BMD was measured at the lumbar spine (L2 - L4) through the dual energy absorptionmetry X-ray (DEXA), using a LUNAR DPX-L densitometer. Preoperatively, all patients showed deficit of the BMD varying from 1 to 7.07 standard deviations (Mean +/- SEM - 2.64 +/- 0.28), considering the mean line of the control curve for healthy children accepted as normal. The BMD deficit was more evident among the females than the males. After treatment there was a significant increment (C2 = 9.19 - p =0.01) of the BMD and 29 percent of the patients (six out of twenty one) were considered without bone mineral deficit. It was concluded that the patients included in this series, who suffer from hepatosplenic mansonic schistosomiasis, showed an important BMD deficit, specially among the females which has had a significant improvement after medical and surgical treatment

Interet de l'association Quinine/Piracetam dans le traitement de la splenomegalie des drepanocytaires homozygotes en zone tropicale

Vingt six sujets drepanocytaires homozygotes porteurs d'une rate volumineuse ont ete traites avec le Piracetam associe a la Quinine administres par voie orale pendant 35 semaines. Au terme de cet essai therapeutique; 21 cas; soit 81 pour cent ont connu une regression remarquable; voire une disparition de la splenomegalie; contre 5 cas d'echec; soit 19 pour cent. Les meilleurs resultats sont obtenus au cours des 16 premieres semaines du traitement et se traduisent par un Index Moyen de Regression de la splenomegalie (I.M.R.S.) tres significatif; variant de 0;75 a 3;3

Reduction of spleen size in a child with hyperreactive malarious splenomegaly (HMS) treatde outside the brazilian endemic area of malaria with only one course of quinine

Relatamos o caso clínico, tratamento e evoluçäo de uma criança com a síndrome da esplenomegalia hiperreativa da malária tratada fora da área endêmica para a malária. A criança apresentava importante esplenomegalia, era procedente de área endêmica para malária, os níveis de imunoglobulinas e de anticorpos antimaláricos estavam elevados e observou-se linfocitose sinusial hepática. A criança näo voltaria mais para a área endêmica de malária, pelo que foi tratada com apenas um curso de quinino apresentando resposta clínica favorável. Esta resposta a um único curso de terapia curativa antimalárica sugere a importância da reinfecçäo com o parasita da malária no desenvolvimento e na maturaçäo desta sídrome

The impact of schistosomiasis mass treatment on hepato-splenomegaly in Napu Valley, Central Sulawesi, Indonesia.

The impact of mass treatment with praziquantel on 241 individuals, 131 males and 110 females in Napu Valley, Indonesia was studied. The pre-treatment overall stool positive cases ranged from 43% to 79%, the highest in the 20-29 age group. Post-treatment follow-up study showed that the cases dropped to 2.5%. Significant reduction was seen in liver rate from 68% to 51% and in the age groups 0-9, 10-19, there was a significant decrease in liver enlargement after treatment, but not significant differences were seen in spleen rate in all age groups. This might be due to the early stage of Schistosoma infection in younger age groups where complete resolution of fibrosis might occur.