[CIS PIDD] [cis-pidd] lymphohistiocytosis vs ALPS?

Filipovich, Alexandra (Lisa) lisa.filipovich at cchmc.org
Wed Feb 18 09:37:47 EST 2015 

What are the inflammatory markers, e.g. sIL2Ra, ferritin?

From: Wilmer Cordova Calderon [mailto:wilmer.cordova at gmail.com]
Sent: Tuesday, February 17, 2015 11:22 PM
To: CIS-PIDD
Subject: Re: [cis-pidd] lymphohistiocytosis vs ALPS?

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<mailto: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<mailto: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<http://www.isn.gob.pe/>
wcordova at insn.gob.pe<mailto:wcordova at insn.gob.pe>
facebook.com/<http://facebook.com/>wilmer.cordova.71
twitter: @wilmer_cordova

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--
Atte
Wilmer Córdova
Inmunología Alergología
Instituto Nacional de Salud del Niño - Perú
http://www.isn.gob.pe<http://www.isn.gob.pe/>
wcordova at insn.gob.pe<mailto:wcordova at insn.gob.pe>
facebook.com/<http://facebook.com/>wilmer.cordova.71
twitter: @wilmer_cordova

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