Lyphoma, vasculitis and arthritis in NBS patient case report Clinical Immunology Department, Russian Children Clinical Hospital.

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Lyphoma, vasculitis and arthritis in NBS patient case report Clinical Immunology Department, Russian Children Clinical Hospital

R.P., born in 1991 One child in the family No family history Microcephalia diagnosed after birth Dismorphic symptoms: receding forehead, prominent midface. Cafeau-lait-spots.

R.P. Lymphoma No serious diseases till 12 years 3 month (Sep. 2003). Sep enlargement of all groups of Peripheral lymph nodes, fever  C. Antibiotics without effect. Admitted in Russian Clinical Children Hospital. NBS syndrome diagnosed: 657-del5 mutation.

R.P. Lymphoma At the time of admission hospital:  peripheral lymph nodes mm  hepar + 3 cm. below rib arch,  spleen +3 cm below rib arch.  Blood count leucopenia – x10 9 /l, neutropenia  increased LDH,  IgA <6.67 mg/dl, IgG-325 mg/dl, IgM-39.8 mg/dl.  7.2% blast cells in bone marrow.

R.P. Lymphoma Lymph node biopsy - Hodgkin disease (two labs) – DAL-HD-90 protocol: Pred 60 мг / м 2, Vcr1,5 мг / м 2 № 1, Adr40 мг / м 2 № 1, Vp мг / м 2 м № 4.  Adverse events: – mucositis, hypertemia, vincristine (oncovin) neuropatia  Treatment: cephtriaxone, vanco, metronidazole, amycacine, G-CSF, ampho-B, IVIG.

R.P. Lymphoma Lymph node biopsy diffuse large B-cell lymphoma, variant T-cell rich 3 group of risk, IV st (cervical, axillar, mediastinal, retroperitoneal lymph nodes and bone marrow involvement) the third lab (prof Dr.Parwaresh, prof Dr. Wacker Germany): All blast cells express CD20, reactive surrounding is CD3 positive. Blast cells are Ki-A10 negative.

R.P. Lymphoma AA(1): MTX 5 g/ m 2, Dexa 10 mg/ m 2 № 5, VP 100 mg/ m 2 № 2, ARA-C 150 mg/ m 2 № 2, IFO 400 mg/ m 2 № 5, Vcr 1,5 mg/ m 2 № 1. Adverse events: delayed MTX clirence (0.24 on 66 houers). day +7 – neutropenic enterocolitis (Enterococcus, Staph), intestinal hemorrhage, pancreatitis Treatment: cefepim+vanco+amicacine, Ampho-B, E-ACA, G-CSF.

R.P. Lymphoma BB(2): Dexa 10 mg/ m 2 № 5, MTX 5 g/ m 2, Adr 25 mg/ m 2 № 2, chp 100 mg/ m 2 № 5, Vcr 1,5 mg/ m 2 № 1. Concominant therapy– cthephepim+fluconazole. Leikovorin. Adverse events:  delayed MTX clirence (4,68 on 42 houers)  Day +7 diarrhea, arterial hypertension. Therapy: cehephepim+vanco+amicacin+AmphoB, GCSF, amlodipin+enalapril.  Day +9 wormiting, bloody diarrhea, oedema, arterial hypertension, strabismus, convulsions. Therapy – carbopenem +vanco +amycacin +AmphoB +metronidazole, leikovorin, G-CSF, dobutamin, nifedipin – clinical and laboratory remission. 1% blast cells in bone marrow.

R.P. Lymphoma CC(3): Dexa 10 mg/ m 2 № 5, Vcr 1,5 mg/ m 2 № 1, VP 100 mg/ m 2 № 3, cytozar high dose - 4 g/ m 2 № 2. Concominant therapy: cephperason/sulbactam +fluconazol. Adverse events:  Day +6 arterial hypertension, sepsis, neutropenic enterocolitis (Candida non-albicans), mucositis. Therapy: cehephepim+vanco+amicacin+AmphoB, GCSF, amlodipin+enalapril.  Day +9 wormiting, bloody diarrhea, oedema, arterial hypertension, strabismus, convulsions. Therapy – carbopenem +vanco +amycacin +AmphoB +metronidazole, leikovorin, G-CSF, dobutamin, nifedipin.

R.P. Lymphoma ОР (4) (Vcr 1.5 мг / м 2 +Pred 40 мг / м 2 ) + Mabthera 500 мг / м 2 (4) + ITLP № 2 (MTX+Dexa+ARA-C). cephepim+vanco+amikacin+valcyclovir, nifedipin, enalapril. Adverse events:  Febrile neutropenia, arterial hypertension. Day +14 Herpes Zoster. Therapy: cepepim+vanco+amicacin+valcyclovir, nifedipin, enalapril.

R.P. Lymphoma COP (5): ( С ph 300 мг / м 2 + Vcr 1.5 мг / м 2 +Pred 40 мг / м 2 ) +Mabthera 500 мг / м 2 + ITLP (MTX+Dexa+Cytozar) Mabthera 500 мг / м COP(6) + Mabthera 500 мг / м 2 + ITLP № 2 (MTX+Dexa+Cytozar). Without adverse reactions.

R.P. Vasculitis+Arthritis Since April erythematouse rash (spots with proliferative component). Skin biopsy shoved vaskulitis. Local treatment with prednisone without effect.

R.P. Vasculitis+Arthritis October 2004 developed polyarthritis. Treatment – prednisone 2 mg/kg with good effect for arthritis and vaskulitis. The dose of prednisone gradually decreased till 5 mg/day.