[CIS PIDD] [cis-pidd] lymphohistiocytosis vs ALPS?
Wilmer Cordova Calderon
wilmer.cordova at gmail.com
Tue Feb 17 23:22:24 EST 2015
Estimado Dr. aquí la tabla donde aparecen más ordenados lo datos de
Linfocitos.
Ahora está más claro.
Algún dato adicional que necesite para brindarlo y gracias por su enlace.
En Perú no tenemos para evaluar el Fas.
Linfos totales
1630
Cd4
349
21%
Cd8
929
57%
Cd3
1331
Linfos T cd45 cd3
1331
81.6%
Cd19
57
3%
NK BRIGTH
0.32%
NK DIM
4.32%
nkt
3.46%
2015-02-16 2:36 GMT-08:00 Nacho Gonzalez <nachgonzalez at gmail.com>:
> Dear Wilmer,
>
> It sounds like ALPS syndrome.
> vit B12? sFASL?
> ALPS can resemble HLH ( http://www.ncbi.nlm.nih.gov/pubmed/24101757)
> Did you check for specific DNT cells? with your data the patient has 21%
> DNT (ab + gd) which is quite high.
>
> Hope this helps
>
> Best regards
>
> Luis Ignacio Gonzalez-Granado
> Immunodeficiencies Unit
> Hospital 12 octubre
> Madrid. Spain
>
>
>
>
> 2015-02-16 11:23 GMT+01:00 Wilmer Cordova Calderon <
> wilmer.cordova at gmail.com>:
>
>> Best regards
>> In Lima Perú find this case that 2 years ago no find a specific
>> diagnostic and child disease is progressing and complicating other
>> non-immune areas. We will be eternally grateful to your help and
>> collaborating diagnosis.
>> Thamk you.
>>
>> Problems:
>> 1. Recurrent pancytopenia associated
>> 2. frequent infections (Pneumonia)
>> background:
>> physiological
>> Psychomotor development 4m, sat at 7-8m, yearling way.
>> Current treatment:
>> 1. multivitamin Forkids
>> 2. Captopril ¼ c / 24h-heart failure secondary portal hypertension.
>> Hospitalizations
>> 1. 2nd 9m pneumonia and anemia Hb 4g%, thrombocytopenia 5d Huancayo
>> 2. 3rd 6m pneumonia and anemia thrombocytopenia hepatosplenomegaly 7d
>> Huancayo
>> 3. Pneumonia anemia thrombocytopenia 6m 4th cervical lymphadenopathy 5d
>> Huancayo
>> 4. 5th pneumonia and anemia thrombocytopenia 5d Huancayo Transfusion PG:
>> 5. 5th (October 2014) purulent lymphadenopathy, anemia, thrombocytopenia
>> CMV, HTP, dilated heart disease and anemia Transfusion 5d PG:
>> infections:
>> 1. Intestinal infections 3v the first year. furoxone antibiotic.
>> 2. SOB 1ª6m.
>> Metabolic studies. Liver biopsy with normal results.
>> Transfusions: PG 4 chances.
>> 02/11/2015
>> October 2014 Table 1. Respiratory, painful cervical lymphadenopathy,
>> content purulent drainage, PCR for CMV 394 copies / ml (positive> 200),
>> pancytopenia (leukocytes 3150, HB 6.6, platelets 107, 000, creat 0.31, FA
>> 1423, reticulocytes 4 %) fever, jaundice.
>> Eco doppler: HT Portal.
>> TEM TAP: hepatosplenomegaly (liver 164mm, 215mm spleen), liquid
>> perisplenic, multiple retroperitoneal lymph nodes, mesenteric, iliac and
>> inguinal up to 15mm.
>> Cervical Doppler ganglion formations with central and peripheral
>> vasculature, reagent aspect, in the context of lymphoproliferative aspect.
>> Pulmonary moderate to severe dilated cardiomyopathy, pericardial
>> effusion, mild systolic dysfunction FE 55% PSAP 62 mmHg HT echocardiography.
>> Treatment: Meropenem and vancomycin; Intravenous Ganciclovir, with
>> improvement of lymphadenopathy and infectious picture.
>> • Biopsy of cervical and inguinal lymphadenopathy: Lymph node with
>> reactive changes predominance of hyperplasia and sinus histiocytosis T zone
>> with occasional eritrofagocitosis. Fibrosis; discrete architectural
>> distortion. CMV neg, CD20 + lymph B, CD3 + in Lymphoma T, DTT neg, CD10 +
>> in follicular centers, Ki67 + cells in replication.
>> Nonspecific reactive lymphoid hyperplasia, immunohistochemistry negative
>> for lymphoid neoplasia, immunoreaction for LMP1 negative.
>> Control abdominal Ecodoppler: higado135mm, spleen 166mm.
>> CMV viral load <200 copies / ml
>> Dilated cardiomyopathy, mild systolic dysfunction FEvi55% HT moderate
>> pulmonary PSAP 47 mmHg
>> 02.11.15 Cold symptoms. Lymphadenopathy multiple cervical, axillary and
>> inguinal approx 2cm phones.
>> Diagnosis
>> 1. Primary Immunodeficiency Vs High.
>> 2. d / c Lymphohistiocytosis recurrent infectious reactive vs ALPS
>> 3. Secondary hypersplenism
>> 4. Hypertension Portal
>> 5. Pulmonary Hypertension
>> 6. dilated cardiomyopathy.
>> 3. CMV infection treated
>>
>> Marzo 14
>> Linfos totales 1630
>> Cd4 349 21%
>> Cd8 929 57%
>> Cd3 1331
>> Linfos T cd45 cd3 1331 81
>> Cd19 57 3%
>> NK 45
>> nkt 3.4
>> Abril 14
>> IgA 168
>> IgG 3177
>> IgM 282
>> IgE 8
>>
>> --
>> *Atte *
>> Wilmer Córdova
>> *Inmunología Alergología*
>> *Instituto Nacional de Salud del Niño - Perú*
>> http://www.isn.gob.pe
>> wcordova at insn.gob.pe
>> facebook.com/wilmer.cordova.71
>> twitter: @wilmer_cordova
>>
>> ---
>>
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>> You are currently subscribed to cis-pidd as: nachgonzalez at gmail.com.
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>>
>
> ---
>
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>
>
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>
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--
*Atte *
Wilmer Córdova
*Inmunología Alergología*
*Instituto Nacional de Salud del Niño - Perú*
http://www.isn.gob.pe
wcordova at insn.gob.pe
facebook.com/wilmer.cordova.71
twitter: @wilmer_cordova
---
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